Publications by authors named "Kevin A Peng"

36 Publications

Cochlear Nerve Canal Stenosis: Association With MYH14 and MYH9 Genes.

Ear Nose Throat J 2021 Jun 8;100(3_suppl):343S-346S. Epub 2021 Mar 8.

House Clinic and House Ear Institute, Los Angeles, CA, USA.

The bony cochlear nerve canal transmits the cochlear nerve as it passes from the fundus of the internal auditory canal to the cochlea. Stenosis of the cochlear nerve canal, defined as a diameter less than 1.0 mm in transverse diameter, is associated with inner ear anomalies and severe to profound congenital hearing loss. We describe an 11-month-old infant with nonsyndromic congenital sensorineural hearing loss with cochlear nerve canal stenosis. Next-generation sequencing revealed heterozygous mutations in and , encoding for the inner ear proteins myosin heavy chain IIA and IIC. The patient's hearing was rehabilitated with bilateral cochlear implantation.
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http://dx.doi.org/10.1177/0145561321996839DOI Listing
June 2021

Radiation-Induced Malignant Peripheral Nerve Sheath Tumor of the Vagus Nerve Following Radiation Treatment of Cervical Paraganglioma.

J Neurol Surg Rep 2020 Oct 31;81(4):e66-e70. Epub 2020 Dec 31.

Division of Neurotology, House Institute, Los Angeles, California, United States.

Radiation-induced sarcoma is a known but rare complication of radiation treatment for skull base paraganglioma. We present the cases of a female patient with multiple paraganglioma syndrome treated with external beam radiation treatment who presented 4 years later with a malignant peripheral nerve sheath tumor of the vagus nerve.
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http://dx.doi.org/10.1055/s-0040-1718408DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7775188PMC
October 2020

Clinical Characteristics of Patients With Papilloma in the External Auditory Canal.

Laryngoscope 2021 05 8;131(5):1132-1137. Epub 2020 Oct 8.

ENT institute and Department of Otorhinolaryngology, Eye & ENT Hospital, State Key Laboratory of Medical Neurobiology, Fudan University, Shanghai, 200031, China.

Objectives: To determine the clinical characteristics of papilloma involving the external auditory canal (PEAC) in a region of China.

Study Design: A retrospective study.

Methods: Demographics, manifestations, imaging results, histopathology, and treatment of 67 patients diagnosed with PEAC in a period of 6 years were analyzed at Shanghai Eye, Ear, Nose and Throat Hospital in China.

Results: PEAC were encountered in patients between the ages of 12 and 82 years (mean 53.8 years). It was more prevalent in men (82%) than in women (18%) (P < .05). The clinical presentation was usually a mass in EAC, aural fullness, and hearing loss. Otoscopic and radiological examination were used together for initial diagnosis and pretreatment planning. Unilateral involvement was more common than bilateral involvement (P < .05). The average time between onset of first symptom and surgical resection and/or biopsy was 6.5 months (range, 0.25-60 months). All patients underwent gross total resection. In 5 patients, (7.5%) carcinoma was detected in the specimen. Fifteen patients (22%) had recurrence; recurrent tumors were detected after an average period of 10 months after surgery (range, 4-24 months).

Conclusion: PEAC is largely a benign lesion with a low risk of malignancy. Optimal management is via gross total resection. However, the risk of recurrence is high, which motivates a need for long-term monitoring.

Level Of Evidence: 4 Laryngoscope, 131:1132-1137, 2021.
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http://dx.doi.org/10.1002/lary.29113DOI Listing
May 2021

Predicting outcome of velopharyngeal surgery in drug-induced sleep endoscopy by traction velum.

Eur Arch Otorhinolaryngol 2021 Mar 24;278(3):821-826. Epub 2020 Aug 24.

House Clinic and House Ear Institute, Los Angeles, CA, 90057, USA.

Background: Outcomes of surgical management of obstructive sleep apnea-hypopnea syndrome (OSAHS) can be difficult to predict preoperatively.

Aims/objectives: To study the effect of applying traction to the velum during drug-induced sleep endoscopy (DISE) in OSAHS patients, and to describe the use of traction velum in predicting surgical success.

Materials And Methods: 41 adult surgical patients with OSA (Friedman tongue position II and III) were evaluated by DISE. All patients subsequently underwent velopharyngeal surgery in the form of uvulopalatopharyngoplasty with tonsillectomy plus barbed reposition pharyngoplasty.

Results: Surgical responders (n = 26, 63.4%) and nonresponders (n = 15, 36.6%) demonstrated no significant differences with regard to preoperative AHI, age, sex, body mass index, and mean/lowest O2 saturation. Responders had a marked decrease in desaturation events (2.96 vs 0.03, p < 0.001) and percentage change in number of desaturation events before/after traction velum (56.7 vs 4.5, p < 0.001). Regression analysis revealed that reduction in mean desaturation events, with traction velum, by a percentage > 26.8% (odds ratio [OR] 1.046; 95% confidence interval [CI] 1.018-1.075; p = 0.001) was the only independent predictor of surgery success.

Conclusions And Significance: OSAHS patients' velopharyngeal surgical outcome can be predicted by measuring the percentage change in the number of oxygen desaturation events before and after traction velum in DISE.
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http://dx.doi.org/10.1007/s00405-020-06292-1DOI Listing
March 2021

Identification of Perinatal Risk Factors for Auditory Neuropathy Spectrum Disorder.

Laryngoscope 2021 03 1;131(3):671-674. Epub 2020 Jul 1.

House Ear Institute, University of Southern California, Los Angeles, California, U.S.A.

Objectives/hypothesis: To identify medical risk factors associated with auditory neuropathy spectrum disorder (ANSD).

Study Design: Retrospective case-control study.

Methods: During a 2-year period (2013-2014) patients with newly diagnosed ANSD were identified at a tertiary care facility. Twenty-two patients (n = 22) were identified aged 0.5 to 8.1 years. There were 15 males and seven females. Sixteen had bilateral, four had left-sided, and two had right-sided ANSD. Two age-matched, side-matched, and gender-matched control groups were then collected. The first group was 22 normal-hearing children (n = 22). The second was 22 children with sensorineural hearing loss (SNHL) (n = 22) who did not meet the criteria for ANSD. The chart of each subject was reviewed for the following five-predictor variables: prematurity, low birth weight, jaundice, use of mechanical ventilation, and administration of ototoxic medications. Analysis of variance was performed to analyze the prevalence of perinatal risk factors among the three groups. Multivariate linear regression was then applied.

Results: When comparing the ANSD group to both the normal-hearing and SNHL groups, the subjects with ANSD had statistically significant higher rates of prematurity, low birth weight, jaundice, and mechanical ventilation. Multiple regression analysis was performed to identify predictors of ANSD compared to each control group individually. Jaundice in the first month of life approached significance when comparing the ANSD group to the normal-hearing group, and was the only medical risk factor found to be statistically significant when comparing the ANSD group to the SNHL group.

Conclusions: A history of neonatal hyperbilirubinemia was significantly more common in children with ANSD compared to children with severe SNHL.

Level Of Evidence: 3 Laryngoscope, 131:671-674, 2021.
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http://dx.doi.org/10.1002/lary.28904DOI Listing
March 2021

An Update on Epidemiology and Management Trends of Vestibular Schwannomas.

Otol Neurotol 2020 03;41(3):411-417

Department of Head and Neck Surgery, University of California, Los Angeles.

Objective: To determine the current epidemiology and management trends for patients with vestibular schwannomas (VS).

Study Design: Retrospective cohort study.

Setting: The Surveillance, Epidemiology, and End Results (SEER) tumor registry.

Patients: The SEER database was queried to identify patients diagnosed with VS from 1973 to 2015. Demographics, patient and tumor characteristics, and treatment methods were analyzed.

Results: A total of 14,507 patients with VS were identified. The mean age at diagnosis was 55 ± 14.9 years. Age-adjusted incidence from 2006 to 2015 was 1.4 per 100,000 per year and remained relatively stable. Incidence across age varied with sex, as younger women and older men had increased incidences comparatively. A higher percentage of patients underwent surgery alone (43%), followed by observation (32%), radiation alone (23%), and combined radiation and surgery (2%). Age 65 and older was associated with observation (odds ratio [OR] 1.417; p = 0.029) whereas age 20 to 39 and 40 to 49 were associated with surgery (OR 2.013 and 1.935; p < 0.001). Older age was associated with radiation. Larger tumor size was associated with surgery and combined treatment. African American patients and American Indian or Alaskan Native patients were more likely to undergo observation than surgery.

Conclusions: The overall incidence of VS is 1.4 per 100,000 per year and has remained relatively stable. There is a trend toward more conservative management with observation, which may be secondary to earlier diagnosis given widespread use of magnetic resonance imaging. Further studies are necessary to investigate differences in disease patterns and disparities in management.
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http://dx.doi.org/10.1097/MAO.0000000000002542DOI Listing
March 2020

Fluctuating Sensorineural Hearing Loss.

Audiol Neurootol 2019 17;24(3):109-116. Epub 2019 Jul 17.

House Clinic and House Ear Institute, Los Angeles, California, USA.

Background: Several otologic conditions can present with fluctuating sensorineural hearing loss, including Ménière's disease, autoimmune inner ear disease, and enlarged vestibular aqueduct. Although these 3 etiologies vary greatly, distinguishing between these conditions at initial presentation can be challenging. Furthermore, initial treatment of these conditions is often similar. In this review, we discuss historical and current perspectives on diagnosis and treatment of these conditions.

Summary: A literature search was performed regarding fluctuating hearing loss, and current treatment of these etiologies of fluctuating hearing loss was summarized. Immediate measures at the onset of acute hearing loss include corticosteroid therapy, while preventative and chronic therapies, which can limit disease severity and frequency, vary based on the specific condition treated. Key Messages: Fluctuating hearing loss can represent a range of pathologies, but the precise etiology may not be clear at initial presentation. Timely treatment and long-term follow-up, along with appropriate diagnostics, are necessary to optimize long-term hearing.
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http://dx.doi.org/10.1159/000500658DOI Listing
April 2020

Linked-read Sequencing Analysis Reveals Tumor-specific Genome Variation Landscapes in Neurofibromatosis Type 2 (NF2) Patients.

Otol Neurotol 2019 02;40(2):e150-e159

The Jackson Laboratory for Genomic Medicine, Farmington, Connecticut.

Hypothesis: We hypothesize that genomic variants including deletions, insertions, inversions, and tandem duplications beyond the changes in tumor suppressor NF2 gene affect gene expression of tumor-specific pathways in vestibular schwannomas (VS) patients with Neurofibromatosis type 2 (NF2), thus contributing to their clinical behavior.

Background: Genomic variation could reconfigure transcription in NF2 transformation process. Therefore, genome-wide high-resolution characterization of structural variants (SV) landscapes in NF2 tumors can expand our understanding of the genes regulating the clinical phenotypes in NF2-associated VS.

Methods: We performed whole-genome haplotype-specific structural variation analysis using synthetic linked reads generated through microfluidics-based barcoding of high molecular weight DNA followed by high-coverage Illumina paired-end whole-genome sequencing from 10 patients' tumors of different growth rates and their matching blood samples.

Results: NF2 tumor-specific deletions and large SVs were detected and can be classified based on their association with tumor growth rates. Through detailed annotation of these mutations, we uncover common alleles affected by these deletions and large SVs that can be associated with signaling pathways implicated in cell proliferation and tumorigenesis.

Conclusion: The genomic variation landscape of NF2-related VS was investigated through whole-genome linked-read sequencing. Large SVs, in addition to deletions, were identified and may serve as modulators of clinical behavior.
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http://dx.doi.org/10.1097/MAO.0000000000002096DOI Listing
February 2019

Revision Surgery for Vestibular Schwannomas.

J Neurol Surg B Skull Base 2018 Dec 9;79(6):528-532. Epub 2018 Apr 9.

House Clinic, Los Angeles, California, United States.

 To describe clinical outcomes of patients undergoing revision surgery for vestibular schwannomas.  Retrospective case series.  Tertiary private neurotologic practice.  Patients who underwent revision surgeries for recurrent/residual vestibular schwannomas between 1985 and 2015.  Degree of resection, facial nerve function, cerebrospinal fluid (CSF) leak.  A total of 234 patients underwent 250 revision surgeries for recurrent/residual vestibular schwannomas. Of these, 86 carried a diagnosis of neurofibromatosis type 2 (NF2). The mean number of prior surgeries was 1.26, and 197 (85%) prior surgeries had been performed elsewhere. The average age at surgery was 43. The most common approach employed at the time of revision surgery was translabyrinthine (87%), followed by transcochlear (6%), middle fossa (5%), and retrosigmoid (2%). Gross total resection was achieved in 212 revision surgeries (85%). Preoperative House-Brackmann facial nerve function was similar in non-NF2 and NF2 groups (mean: 2.7). Mean postoperative facial nerve function at last follow-up was 3.8 in the non-NF2 group and 3.9 in the NF2 group. History of radiation and the extent of resection were not associated with differences in facial nerve function preoperatively or postoperatively. CSF leaks occurred after 21 surgeries (8%), and six (2%) patients required reoperation.  This is the largest series of revision surgery for vestibular schwannomas to date. Our preferred approach is the translabyrinthine craniotomy, which can be readily modified to include the transcochlear approach for improved access. CSF leak rate slightly exceeds that of primary surgery, and gross total resection is achievable in the vast majority of patients.
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http://dx.doi.org/10.1055/s-0038-1635256DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6239869PMC
December 2018

Pearls for the middle fossa approach in acoustic neuroma surgery.

Curr Opin Otolaryngol Head Neck Surg 2018 Oct;26(5):276-279

House Clinic, Los Angeles, California, USA.

Purpose Of Review: To discuss the use of the middle fossa craniotomy for resection of vestibular schwannomas; to present pearls of and modifications to the approach.

Recent Findings: The middle fossa craniotomy allows for hearing preservation in the resection of intracanalicular vestibular schwannomas. Over recent years, the approach has been modified to address larger tumors with a limited cerebellopontine angle component. Positive identification of the superior semicircular canal allows for rapid exposure of the internal auditory canal (IAC). Removal of cerebrospinal fluid from the posterior fossa during exposure of the IAC allows for removal of the middle fossa retractor; reinforced silicone sheeting is used to protect the middle fossa dura during further drilling. The use of the endoscope has allowed for more complete dissection at the fundus of the IAC, including tumors lateral to the transverse crest.

Summary: Technical modifications to the middle fossa craniotomy have allowed for a shorter duration of temporal lobe retraction intraoperatively and more complete resection of tumors with fundal involvement.
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http://dx.doi.org/10.1097/MOO.0000000000000479DOI Listing
October 2018

Cochlear implantation and auditory brainstem implantation in neurofibromatosis type 2.

Laryngoscope 2018 09 24;128(9):2163-2169. Epub 2018 Mar 24.

House Clinic, Los Angeles, California, U.S.A.

Objectives/hypothesis: To report a series of patients with neurofibromatosis type 2 (NF2), where each patient underwent both cochlear implantation and auditory brainstem implantation for hearing rehabilitation, and to discuss factors influencing respective implant success.

Study Design: Retrospective case series.

Methods: Ten NF2 patients with both cochlear implantations and auditory brainstem implantations were retrospectively reviewed. Speech testing for auditory brainstem implants (ABIs) and cochlear implants (CIs) was performed separately. Scores at last follow-up were obtained for Iowa vowels and consonants, Northwestern University Children's Perception of Speech (NU-CHIPS), and City University of New York (CUNY) sentences.

Results: Mean age at time of implant was 37 years for cochlear implantation and 40 years for auditory brainstem implantation (P = .790, t test). Nine of 10 patients had a CI and ABI on contralateral sides, and one had both devices on the same side. Mean duration of deafness in the implanted ear was 4.3 years for both cochlear implantation and auditory brainstem implantation (P = .491, t test). Follow-up range was 1 to 28 years. CI performance on NU-CHIPS was 32% to 100%, and sound + lip-reading CUNY was 56% to 100%. Four patients experienced an eventual decline in CI function to unusable levels. ABI performance on NU-CHIPS was 40% to 80%, and sound + lip-reading CUNY was 38% to 94%. There was no notable decline in ABI function over time.

Conclusions: If the cochlear nerve is intact, cochlear implantation can be an effective strategy for hearing rehabilitation in NF2. However, a significant proportion experience a decline in CI performance related to growing vestibular schwannoma or tumor treatment. Auditory brainstem implantation remains the standard option for surgical hearing rehabilitation in NF2, but peak performance is generally lower than that achievable with cochlear implantation.

Level Of Evidence: 4. Laryngoscope, 128:2163-2169, 2018.
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http://dx.doi.org/10.1002/lary.27181DOI Listing
September 2018

Schwartze sign.

Ear Nose Throat J 2018 Mar;97(3):54

House Clinic, Los Angeles, CA, USA.

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http://dx.doi.org/10.1177/014556131809700315DOI Listing
March 2018

A case of squamous cell carcinoma of the nasal cavity in a patient with granulomatosis with polyangiitis (Wegener granulomatosis).

Ear Nose Throat J 2018 Jan-Feb;97(1-2):E37-E41

Department of Head and Neck Surgery, Ronald Reagan UCLA Medical Center, 10833 Le Conte Ave., 62-132 CHS, Los Angeles, CA 90095, USA.

We report a rare case of squamous cell carcinoma (SCC) of the nasal cavity arising in a patient with granulomatosis with polyangiitis (GPA). The patient was a 35-year-old man who had been diagnosed 15 years earlier with GPA and treated medically for sinonasal, pulmonary, and renal involvement. He presented to us with left-sided orbital and cheek pain and nasal obstruction. Endoscopy detected a friable, exophytic mass that involved the left lateral nasal wall and septum. Biopsy analysis identified the mass as an SCC. A definitive endoscopic resection was performed, followed by chemoradiation, but the patient exhibited progression of disease 2 months after the cessation of therapy. He then underwent an open craniofacial resection and a second round of chemoradiation. At 7 months of follow-up, he remained disease-free. Sinonasal symptoms in GPA are consistent with those in chronic rhinosinusitis, but the presence of unilateral symptoms may suggest a neoplastic process. Immunosuppressants are implicated in the pathophysiology of this malignancy, but equally plausible is the oncogenic role of chronic inflammation.
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http://dx.doi.org/10.1177/0145561318097001-209DOI Listing
September 2018

Characteristics of electrically evoked auditory brainstem responses in patients with cochlear nerve canal stenosis receiving cochlear implants.

Int J Pediatr Otorhinolaryngol 2018 Jan 20;104:98-103. Epub 2017 Oct 20.

House Clinic, 2100 W 3rd St. Suite 111, Los Angeles, CA 90057, USA.

Objective: To explore the characteristics of the electrically evoked auditory brainstem responses (EABR) in children with cochlear nerve canal stenosis (CNCs) following cochlear implantation (CI), and the EABR thresholds in children with stenotic versus normal cochlear nerve canals.

Method: Sixteen children with profound sensorineural hearing loss were included in this study: 8 with CNCs (CNCs group) and 8 with normal cochlear nerve canals (control group). All children underwent cochlear implantation with full insertion of all electrodes. EABR was performed 6 months postoperatively in both groups.

Results: The EABR extraction rate was 100% in children with normal cochlear nerve canals and only 50% in children with CNCs. EABR thresholds were significantly higher in children with CNCs of electrodes No. 11and 22 than in children with normal cochlear nerve canals (P < 0.05 for both comparisons). There was no significant difference in EABR thresholds among electrode No. 1, 11 and 22 in CNCs group (P > 0.05 for all comparisons); while in the control group, the EABR threshold at electrode No 22 was lower than those at both electrodes No. 11 and 1 (P < 0.05 for both comparisons), and the EABR threshold at electrode No. 11 was also lower than that at electrode No. 1 (P < 0.05).

Conclusion: The EABR thresholds in children with normal cochlear nerve canals vary according to the different locations of electrodes in the cochlea; while in children with CNCs, there was no significant difference among different electrode locations. The EABR thresholds in CNCs children were higher than those of children with normal cochlear nerve canals at electrode 11 and 22.
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http://dx.doi.org/10.1016/j.ijporl.2017.10.029DOI Listing
January 2018

Otolaryngic manifestations of Cushing disease.

Ear Nose Throat J 2017 Aug;96(8):E28-E30

Department of Head and Neck Surgery, David Geffen School of Medicine at UCLA, 10833 Le Conte Ave., CHS 62-132, Los Angeles, CA 90095-1624, USA.

Cushing disease is a relatively rare cause of Cushing syndrome secondary to a hyperfunctioning pituitary adenoma. In addition to signs and symptoms of hypercortisolism, Cushing disease may present with diverse otolaryngic manifestations, which may guide diagnosis and management. We performed a retrospective chart review of patients who were found to have Cushing disease and who underwent transnasal transsphenoidal surgery for pituitary adenomas between January 1, 2007, and July 1, 2014, at a tertiary academic medical center. There were 37 consecutive patients in this series with Cushing disease caused by a pituitary adenoma. Fifteen (41%) patients complained of visual changes. Five (14%) patients suffered from obstructive sleep apnea. Four (11%) patients had thyroid disease. Other symptoms included hearing loss, vertigo, tinnitus, epistaxis, dysphagia, and salivary gland swelling. Although Cushing disease traditionally presents with classic "Cushingoid" systemic features, it also may present with various otolaryngic manifestations. A thorough workup by otolaryngologists is critical in the comprehensive management of these patients.
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http://dx.doi.org/10.1177/014556131709600808DOI Listing
August 2017

Cochlear Nerve Aplasia and Hypoplasia: Predictors of Cochlear Implant Success.

Otolaryngol Head Neck Surg 2017 09 4;157(3):392-400. Epub 2017 Jul 4.

1 House Clinic, Los Angeles, California, USA.

Objective To identify factors predicting performance outcomes following cochlear implantation in patients with cochlear nerve aplasia or hypoplasia. Data Sources Individual patient data extracted from published case series and reports. Review Methods The MEDLINE database, Cochrane Library, Embase, Web of Science, and Google Scholar were queried for "cochlear implant" in conjunction with "aplasia" or "hypoplasia" between 1985 and 2015. Eighteen studies were included describing 97 subjects with individual postimplant auditory data. Postimplant performance was categorized as follows: level 1, nonstimulation/minimal detection; level 2, improved detection; level 3, closed-set speech perception; or level 4, open-set speech perception. The subjects achieving speech perception (levels 3 and 4) were descriptively compared with those who did not. Results Subjects with a hypoplastic cochlear nerve on magnetic resonance imaging had higher reported rates of achieving speech perception than those with an aplastic nerve. Subjects with syndromic medical comorbidities had higher reported rates of nonstimulation than nonsyndromic subjects. The data showed that some children with an aplastic cochlear nerve or those with partial electrode insertion could obtain levels of speech discrimination. Reporting of patient characteristics and auditory outcomes was extremely variable across studies. Conclusion As previously shown, cochlear implant in patients with cochlear nerve aplasia or hypoplasia can provide meaningful hearing for select patients. The current study suggests that presence of a cochlear nerve on magnetic resonance imaging and lack of comorbid medical syndrome are associated with better auditory outcomes in such patients. Future efforts to report individual data in a consistent manner may allow better determination of predictive factors.
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http://dx.doi.org/10.1177/0194599817718798DOI Listing
September 2017

Angiosarcoma of the tongue: A case series and literature review.

Am J Otolaryngol 2017 Jul - Aug;38(4):475-478. Epub 2017 Apr 21.

Department of Head and Neck Surgery, David Geffen School of Medicine at UCLA, Los Angeles, California, USA. Electronic address:

Purpose: Angiosarcoma of the tongue is an exceedingly rare malignancy of the head and neck. Such lesions can be primary in nature or occur in a previously irradiated field. We examine a series of cases with relation to clinical presentation, diagnosis, management, and outcomes.

Materials And Methods: Retrospective chart review of all patients with angiosarcoma of the tongue at a tertiary academic institution yielded a single case between 2005 and 2016. The MEDLINE database was additionally searched for all case series or reports of angiosarcoma arising in the tongue, and pertinent clinical data were extracted.

Results: The clinical presentation, disease course, and management of a patient with angiosarcoma of the tongue are presented. Institutional and literature search yielded a total of eight patients with angiosarcoma of the tongue. The most common primary sites were dorsal and lateral oral tongue. Treatment consisted of surgical resection in 63% of cases with adjuvant therapy administered in 75% of cases. Follow-up times varied per patient, but 63% had persistent or recurrent disease and 67% died of or with disease within two years of index presentation.

Conclusion: Angiosarcoma of the tongue is a rare and highly aggressive tumor, accounting for fewer than 1% of all head and neck malignancies. The mainstay of treatment is surgical resection with negative margins followed by adjuvant chemoradiation for high-risk features. Due to rarity of the disease, consensus on optimal treatment approach is lacking, and multi-center prospective studies would be helpful to set clinical guidelines.
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http://dx.doi.org/10.1016/j.amjoto.2017.04.013DOI Listing
May 2018

Sinonasal quality of life outcomes following laser treatment of epistaxis related to hereditary hemorrhagic telangiectasia.

Lasers Med Sci 2017 Apr 24;32(3):527-531. Epub 2017 Jan 24.

Department of Head and Neck Surgery, David Geffen School of Medicine at the University of California Los Angeles (UCLA), 10833 Le Conte Ave, CHS 62-132, Los Angeles, CA, 90095, USA.

Hereditary hemorrhagic telangiectasia (HHT) is an inherited vascular disorder which manifests as recurrent, episodic, and potentially debilitating epistaxis. In this study, we aim to (1) characterize baseline sinonasal symptoms for HHT patients and to (2) analyze changes in sinonasal symptoms before and after laser surgical treatment for HHT. We performed a retrospective chart review of sinonasal outcome test-22 (SNOT-22) scores before and after one or more laser surgical treatments for HHT-related epistaxis between January 1, 2010 and December 1, 2015 at a tertiary academic medical center with an HHT Foundation-approved Center of Excellence. Preoperative and all subsequent postoperative SNOT-22 scores (short-term, <45 days and long-term, ≥45 days) were compared. Twenty consecutive HHT patients underwent 51 laser surgeries for recurrent epistaxis. Mean preoperative, short-term postoperative, and long-term postoperative SNOT-22 scores were 34.6 ± 5.4, 33.9 ± 5.5, and 18.8 ± 4.6, respectively. When analyzing subcategory scores, there was a significant improvement in the rhinologic domain from short-term to long-term postoperatively (13.5 vs. 7.3; p = 0.004), in the non-rhinologic otolaryngic domain from short-term to long-term postoperatively (2.8 vs. 1.7; p = 0.014), and in the psychological domain from preoperative and short-term postoperative to long-term postoperatively (12.2 and 10.0 vs. 6.0; p = 0.015 and 0.01, respectively). Following laser surgery for HHT-related epistaxis, patients' rhinologic symptoms worsened on the short run postoperatively but improved over time. The main benefit of laser treatment appears to be long-term improvement in psychological factors. This study once again underscores the important role of the otolaryngologist in managing sinonasal manifestations of HHT.
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http://dx.doi.org/10.1007/s10103-017-2144-7DOI Listing
April 2017

Facial nerve stimulation following cochlear implantation for X-linked stapes gusher syndrome leading to identification of a novel POU3F4 mutation.

Int J Pediatr Otorhinolaryngol 2016 Dec 6;91:121-123. Epub 2016 Oct 6.

Department of Head and Neck Surgery, David Geffen School of Medicine, University of California Los Angeles, Los Angeles, CA, USA. Electronic address:

We report a case of a nine-year-old male who presented with facial nerve stimulation four years after cochlear implantation. Computed tomography was performed revealing a dilated internal auditory meatus and the cochlear implant electrode was found to be protruding into the fallopian canal at the level of the geniculate ganglion. Subsequent genetic analysis demonstrated X-linked deafness type 2 (DFNX2) caused by a novel c.769C > T nucleotide change in the POU domain, class 3, transcription factor 4 gene (POU3F4). Inactivation of electrodes 1 and 19-21 successfully abated facial nerve stimulation.
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http://dx.doi.org/10.1016/j.ijporl.2016.10.003DOI Listing
December 2016

Tinnitus Suppression After Auditory Brainstem Implantation in Patients With Neurofibromatosis Type-2.

Otol Neurotol 2017 01;38(1):118-122

*Division of Otolaryngology-Head and Neck Surgery, University of Connecticut School of Medicine, Farmington, Connecticut †House Clinic and House Ear Institute, Los Angeles, California ‡Department of Otolaryngology-Head and Neck Surgery, William Beaumont Army Medical Center, El Paso, Texas §House Clinic, Neurosurgery, Los Angeles, California ||Huntington Medical Research Institutes, Pasadena, California.

Objective: To evaluate whether an auditory brainstem implant (ABI) can impact levels of tinnitus in neurofibromatosis type-2 (NF2) patients who have undergone translabyrinthine craniotomy for vestibular schwannoma (VS) removal and to evaluate the burden of tinnitus in these patients.

Study Design: A retrospective case series and patient survey.

Setting: Tertiary neurotologic referral center.

Patients: NF2 patients who underwent translabyrinthine removal of VS and ABI placement between 1994 and 2015.

Interventions: A survey, retrospective review and two validated tinnitus handicap questionnaires (tinnitus handicap inventory [THI] and tinnitus visual analogue scale [VAS]) were used to characterize the degree of tinnitus in NF2 patients and whether an ABI can alter tinnitus levels.

Main Outcome Measures(s): Survey results, THI and VAS scores.

Results: One hundred twelve ABI users were contacted and 43 patients (38.3)% responded to our survey. Tinnitus was reported in 83.7% of patients. The THI score for responders was 17.8 ± 20.5 standard deviation (SD). For survey participants, the ABI reduced tinnitus levels (mean VAS: Off = 3.5; On 1-h = 2.1; p = 0.048). For patients who subjectively reported that the ABI reduced tinnitus loudness, tinnitus levels were immediately reduced on ABI activation and after 1 hour of use (mean VAS: Off = 4.8; On = 2.4; On 1-h = 1.8; p < 0.01). Suppression did not continue after the device was turned off. Audiological performance with the ABI did not correlate with tinnitus suppression.

Conclusion: NF2 patients who have undergone removal of VS have a significant tinnitus handicap and benefit from tinnitus suppression through utilization of an ABI possibly through masking or electrical stimulation of the auditory brainstem.
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http://dx.doi.org/10.1097/MAO.0000000000001230DOI Listing
January 2017

Atrophy of the Stria Vascularis.

Otol Neurotol 2016 Feb;37(2):e9-11

*House Clinic †The Otoneurology and House Histologic Temporal Bone Laboratory of UCLA, Los Angeles, California, U.S.A.

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http://dx.doi.org/10.1097/MAO.0000000000000935DOI Listing
February 2016

Laryngeal oncocytic cystadenomas masquerading as laryngoceles.

Am J Otolaryngol 2016 Jan-Feb;37(1):17-21. Epub 2015 Sep 10.

Department of Head and Neck Surgery, David Geffen School of Medicine at UCLA, Los Angeles, CA, USA; Jonsson Comprehensive Cancer Center, David Geffen School of Medicine at UCLA, Los Angeles, CA, USA. Electronic address:

Objective: To describe an experience with laryngeal oncocytic cystadenomas and review the published literature regarding this uncommon diagnosis.

Methods And Results: A clinical review of patients presenting with cystic laryngeal masses in an urban academic medical center between January and December 2013 was performed. Three patients, two female and one male, with a mean age of 68 years, were diagnosed with oncocytic cystadenomata of the larynx. Major presenting symptoms included dysphonia, globus, and ipsilateral otalgia. Endoscopic examinations revealed a cystic structure arising from varied subsites of the larynx: laryngeal ventricle, aryepiglottic fold, and pre-epiglottic space. Cross-sectional radiographic imaging was obtained in each case. The patients were treated with transoral (CO2) laser microsurgery (TLM). In all three cases, pathological analysis revealed oncocytic cystadenoma with clear margins.

Conclusions: Oncocytic cystadenoma is a rare entity of the larynx predominantly affecting elderly patients. Clinical presentation and imaging may suggest the diagnosis of an internal laryngocele. Complete excision is both diagnostic and therapeutic, and typically can be achieved using TLM. When clear margins are obtained, no adjuvant therapy is indicated. Although laryngoceles and malignancies are more commonly encountered, oncocytic cystadenomas should remain in the differential of cystic laryngeal masses.
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http://dx.doi.org/10.1016/j.amjoto.2015.09.010DOI Listing
September 2016

Sinonasal outcomes in obstructive sleep apnea syndrome.

Laryngoscope 2015 Nov 24;125(11):2617-20. Epub 2015 Jun 24.

Department of Head and Neck Surgery, David Geffen School of Medicine at the University of California, Los Angeles Medical Center, Los Angeles, California, U.S.A.

Objectives/hypothesis: Obstructive sleep apnea syndrome (OSAS) is a common adult and pediatric sleep disorder, which, if left untreated, is associated with severe medical consequences. Otolaryngologists are often challenged by the impact of OSAS on sinonasal symptoms, as well as by the contribution of primary nasal disorders to exacerbation of OSAS. The objectives of this study are to explore the relationship between patients with known OSAS and quality-of-life outcomes as measured by 22-iem Sino-Nasal Outcome Test (SNOT-22) questionnaire scores.

Study Design: Retrospective chart review.

Methods: Patients with a polysomnographic diagnosis of OSAS completed SNOT-22 questionnaires. SNOT-22 questionnaire items were subcategorized into rhinologic, nonrhinologic otolaryngic, sleep, and psychological symptoms. Total, individual, and subcategorized SNOT-22 scores were correlated with apnea-hypopnea index (AHI), body mass index (BMI), lowest oxygen desaturation, and sleep efficiency using Spearman rank correlation.

Results: A total of 30 consecutive OSAS patients were included in the analysis. BMI was positively correlated with nonrhinologic otolaryngic symptoms (P=.03), specifically ear fullness (P=.02) and facial pain/pressure (P=.04). AHI was negatively correlated with postnasal drip (P=.02). Lowest oxygen saturation was correlated with reduced productivity (P=.03). Sleep efficiency was negatively correlated with difficulty falling asleep (P=.03) and feelings of frustration (P=.01), with a trend toward significant correlation with overall sleep symptoms (P=.08).

Conclusions: Several sinonasal complaints appear to be correlated with OSAS severity. As such, otolaryngologists should utilize a comprehensive approach to the care of the OSAS patient, with special attention to sinonasal factors.

Level Of Evidence: NA.
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http://dx.doi.org/10.1002/lary.25411DOI Listing
November 2015

A swallow preservation protocol improves function for veterans receiving chemoradiation for head and neck cancer.

Otolaryngol Head Neck Surg 2015 May 31;152(5):863-7. Epub 2015 Mar 31.

Department of Head and Neck Surgery, David Geffen School of Medicine at UCLA, Los Angeles, California, USA Department of Surgery, Veterans Affairs Greater Los Angeles Healthcare System, Los Angeles, California, USA Research Service, Veterans Affairs Greater Los Angeles Healthcare System, Los Angeles, California, USA.

Objective: Determine the efficacy of a swallow preservation protocol (SPP) on maintaining swallow function in patients undergoing chemoradiation (CRT) or radiation therapy alone (RT) for head and neck squamous cell carcinoma (HNSCC).

Study Design: Retrospective case series.

Setting: Veterans Affairs medical center.

Subjects And Methods: Patients treated with CRT or RT for HNSCC between February 2006 and November 2013 were studied. Those enrolled in the SPP participated in swallowing, jaw, and tongue exercises during cancer therapy. The comparator group received no swallowing intervention during CRT. A previously described functional outcome swallowing scale (FOSS; 0 = no symptoms and 5 = nonoral feeding for all nutrition) was used to quantify dysphagia prior to and at the completion of cancer therapy, and an analysis was performed to compare swallowing function.

Results: Forty-one (all male; mean age, 66 years) and 66 patients (all male; mean age, 61 years) were included in the SPP and comparator groups, respectively. In the SPP group, mean pre- and posttreatment FOSS scores were 2.2 and 2.2, respectively, while the corresponding scores in the comparator group were 1.8 and 2.7, respectively, with posttreatment FOSS scores being significantly worse than pretreatment FOSS scores in the comparator group only.

Conclusion: Patients enrolled in the SPP demonstrated preserved swallowing function over the course of cancer treatment compared with a comparator group. This confirms the importance of early evaluation and intervention for dysphagia prior to and during CRT or RT alone.
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http://dx.doi.org/10.1177/0194599815575508DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4768734PMC
May 2015

Basaloid squamous cell carcinoma of the maxilla: Report of a case and literature review.

Am J Otolaryngol 2015 May-Jun;36(3):402-7. Epub 2015 Jan 30.

Department of Head and Neck Surgery, University of California, Los Angeles (UCLA) Medical Center, Los Angeles, CA; Jonsson Comprehensive Cancer Center, UCLA Medical Center, Los Angeles, CA; UCLA Head and Neck Cancer Program, UCLA Medical Center, Los Angeles, CA. Electronic address:

Purpose: Basaloid squamous cell carcinoma (BSCC) is a rare variant of squamous cell carcinoma characterized by a highly aggressive clinical course. Though typically found in the larynx, oropharynx, and hypopharynx, we report a rare case of BSCC originating in the maxillary sinus in an otherwise healthy 32-year-old male.

Materials And Methods: Single case report of a patient with BSCC of the maxillary sinus and retrospective chart review of all cases of BSCC of the maxilla at a single academic institution between January 1, 1986 and December 31, 2013. The MEDLINE database was additionally queried for all case series or reports of BSCC arising in the maxilla, and pertinent clinical data were extracted.

Results: The clinical presentation, disease course, and management of a patient with BSCC of the maxilla are presented. In this recent case, the patient presented with persistent alveolar pain and a nonhealing tooth infection. Radiographic studies demonstrated a large necrotic mass in the left maxillary sinus that was biopsy-proven as BSCC. The patient underwent surgical resection followed by postoperative radiation without complications.

Conclusions: BSCC of the maxilla is a rare oncologic entity that may progress to late disease stage without obvious clinical signs or symptoms. Optimal treatment involves complete surgical resection followed by postoperative.
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http://dx.doi.org/10.1016/j.amjoto.2015.01.015DOI Listing
February 2016

Acromegaly: otolaryngic manifestations following pituitary surgery.

Am J Otolaryngol 2015 Jul-Aug;36(4):521-5. Epub 2015 Mar 7.

Department of Head and Neck Surgery, David Geffen School of Medicine at the University of California, Los Angeles (UCLA) Medical Center, Los Angeles, CA. Electronic address:

Objectives: Acromegalics present with a wide range of otolaryngic symptoms, including rhinosinusitis, changes in facial appearance, obstructive sleep apnea (OSA), and voice disturbances. Treatment typically involves transnasal-transsphenoidal (TNTS) resection of the offending pituitary adenoma. In this study, we identify the prevalence of otolaryngic symptoms of acromegalic patients, and evaluate Sinonasal Outcome Test (SNOT-22) scores preceding and following pituitary resection.

Design: Retrospective chart review.

Setting: Tertiary academic medical center.

Participants: Patients diagnosed with acromegaly who underwent surgical resection of a growth-hormone secreting pituitary adenoma between August 2010 and September 2013.

Main Outcome Measures: Subjects were asked to complete questionnaires detailing otolaryngic symptoms as well as SNOT-22 surveys before and after TNTS surgery. A Student's t-test was used to compare preoperative and postoperative SNOT-22 scores.

Results: Twenty-five patients underwent pituitary surgery for acromegaly. Acromegalic patients were found to have macroglossia (60%), OSA or sleep-disordered breathing (52%), thyroid neoplasia (20%), hearing loss/tinnitus (20%), sinonasal symptoms (16%), and parathyroid pathology (8%). Differences in preoperative and postoperative SNOT-22 scores were not statistically significant.

Conclusion: Acromegalics present with assorted otolaryngic complaints. Routine screening of all acromegalics with sleep evaluations (for both surgical and perioperative planning), thyroid ultrasound, and audiologic testing should be strongly considered.
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http://dx.doi.org/10.1016/j.amjoto.2015.03.001DOI Listing
March 2016

Delayed intravenous contrast-enhanced 3D FLAIR MRI in Meniere's disease: correlation of quantitative measures of endolymphatic hydrops with hearing.

Clin Imaging 2015 Jan-Feb;39(1):26-31. Epub 2014 Oct 16.

Department of Head and Neck Surgery, David Geffen School of Medicine at UCLA, Los Angeles, CA USA.

Objective: Using three-dimensional fluid-attenuated inversion recovery magnetic resonance imaging (3D-FLAIR MRI), our goal was to correlate quantifiable measures of endolymphatic hydrops (EH) with auditory function in the setting of Meniere's disease (MD).

Materials And Methods: Forty-one ears were analyzed in 21 subjects (12 ears with MD, 29 without MD). Vestibular endolymphatic space size measurements obtained with two different techniques were referenced against clinical data.

Results: EH was better evaluated on 3D maximum intensity projections (MIPs) than on two-dimensional (2D) images. Using MIPs, quantitative assessments EH correlated with severity of hearing impairment.

Conclusion: 3D MIPs were superior to 2D images for evaluating EH in the setting of MD.
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http://dx.doi.org/10.1016/j.clinimag.2014.09.014DOI Listing
June 2015

Temporal bone fracture causing superior semicircular canal dehiscence.

Case Rep Otolaryngol 2014 10;2014:817291. Epub 2014 Sep 10.

Department of Head and Neck Surgery, David Geffen School of Medicine at the University of California, Los Angeles, 10833 Le Conte Avenue, CHS 62-132, Los Angeles, CA 90095, USA.

Importance. Superior semicircular canal dehiscence (SCD) is a third window lesion of the inner ear causing symptoms of vertigo, autophony, tinnitus, and hearing loss. A "two-hit" hypothesis has traditionally been proposed, whereby thinly developed bone overlying the superior canal is disrupted by a sudden change in intracranial pressure. Although the symptoms of SCD may be precipitated by head injury, no previous reports have described a temporal bone fracture directly causing SCD. Observations. Two patients sustained temporal bone fractures after closed head trauma, and developed unilateral otologic symptoms consistent with SCD. In each instance, computed tomography imaging revealed fractures extending through the bony roof of the superior semicircular canal. Conclusions and Relevance. Temporal bone fractures, which are largely treated nonoperatively, have not previously been reported to cause SCD. As it is a potentially treatable entity, SCD resulting from temporal bone fracture must be recognized as a possibility and diagnosed promptly if present.
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http://dx.doi.org/10.1155/2014/817291DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4176638PMC
October 2014

Utility of the transnasal esophagoscope in the management of chemoradiation-induced esophageal stenosis.

Ann Otol Rhinol Laryngol 2015 Mar 15;124(3):221-6. Epub 2014 Sep 15.

Department of Head and Neck Surgery, David Geffen School of Medicine at UCLA, Los Angeles, California, USA

Objective: This study aimed to describe management of esophageal stenosis after chemoradiation therapy for head and neck squamous cell carcinoma (HNSCC), with particular emphasis on techniques and outcomes with the use of the transnasal esophagoscope (TNE) in the office as well as operating room settings.

Methods: Retrospective analysis of all patients with esophageal stenosis following head and neck cancer radiation, with or without chemotherapy, and managed with TNE-assisted esophageal dilation over a 5-year period. Preoperative and postoperative swallowing function were assessed objectively with the Functional Outcome Swallowing Scale (FOSS; ranging from score 0, a normal diet, to score 5, complete dependence on nonoral nutrition).

Results: Twenty-five patients met inclusion criteria. The mean pretreatment FOSS score was 4.4, whereas the mean posttreatment FOSS score was 2.7 (Wilcoxon signed-rank test, P<.001). Prior to dilation, 16 patients were completely gastrostomy-tube dependent (FOSS 5), of whom 12 (75%) were able to tolerate oral nutrition for a majority of their diet following treatment according to our protocol. No complications were noted.

Conclusion: Dysphagia following chemoradiation therapy for HNSCC is often related to esophageal stenosis. With the aid of TNE, we have developed a successful treatment strategy for esophageal stenosis with improved success rates.
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http://dx.doi.org/10.1177/0003489414550858DOI Listing
March 2015

Head and neck sarcomas: analysis of the SEER database.

Otolaryngol Head Neck Surg 2014 Oct 18;151(4):627-33. Epub 2014 Aug 18.

Department of Head and Neck Surgery, David Geffen School of Medicine, University of California, Los Angeles, California, USA.

Objective: To summarize the epidemiology of sarcomas occurring in the head and neck and identify prognostic factors for patient survival.

Study Design And Setting: Cross-sectional analysis of the National Cancer Institute's Surveillance, Epidemiology and End Results (SEER) program.

Methods: The SEER 18 registries, comprising sarcoma diagnoses made from 1973 to 2010, were queried for sarcomas arising in the head and neck. Pediatric and adult patients were analyzed separately, and multivariate and propensity-matched analyses were performed to identify predictors of disease-specific survival.

Results: In all, 11,481 adult cases and 1244 pediatric cases were identified. In adults, the most common histologic subtypes were malignant fibrous histiocytoma (MFH), Kaposi sarcoma, and hemangiosarcoma, while in the pediatric cohort, the most common histologic subtypes were rhabdomyosarcoma, MFH, and osteosarcoma. Cause-specific 2-, 5-, and 10-year survival rates were 76%, 66%, and 61% for adults and 84%, 73%, and 71% for pediatric patients. Multivariate analysis performed for adults revealed that male gender, absence of radiation therapy, and stage I disease were associated with improved cause-specific survival reaching statistical significance. However, a propensity-matched model demonstrated no significant difference in cause-specific survival between patients who received radiation and those who did not.

Conclusion: Sarcomas, a heterogeneous group of malignant mesenchymal tumors, are uncommonly found in the head and neck. This study represents the largest analysis of patients with head and neck sarcomas in the literature and demonstrates the impact of age, gender, primary site, histology, and radiation status on overall prognosis.
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http://dx.doi.org/10.1177/0194599814545747DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4598178PMC
October 2014