3,182 results match your criteria auditory rare


Reversible splenial lesion syndrome in sisters with sensorineural deafness as the first manifestation.

Heliyon 2021 May 26;7(5):e07057. Epub 2021 May 26.

Department of Neurology, Second Hospital of Hebei Medical University, Shijiazhuang, Hebei, 050000, China.

Reversible splenial lesion syndrome (RESLES) is a rare clinico-radiological disorder syndrome with unclear pathophysiology. Here, two sisters with sensorineural deafness as the chief complaint diagnosed with RESLES was reported. Although the sisters had the disease successively, they were divided into two types on imaging by isolated lesions of splenium of the corpus callosum (SCC) and extensive lesions of the corpus callosum. Read More

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Whole-Exome Sequencing Reveals a Rare Variant of Gene Causing Congenital Non-syndromic Hearing Loss Among Large Muslim Families Favoring Consanguinity.

Front Genet 2021 25;12:641925. Epub 2021 May 25.

Human Genetics and Toxicology Laboratory, Section of Genetics, Department of Zoology, Aligarh Muslim University, Aligarh, India.

Non-syndromic hearing loss (NSHL) is one of the most frequent auditory deficits in humans characterized by high clinical and genetic heterogeneity. Very few studies have reported the relationship between (Locus: DFNB9) and hereditary hearing loss in India. We aimed to decipher the genetic cause of prelingual NSHL in a large affected Muslim consanguineous families using whole-exome sequencing (WES). Read More

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Reconstruction of the external auditory canal: The tragal flap revisited and review of contemporary reconstructive techniques.

Am J Otolaryngol 2021 May 31;42(6):103094. Epub 2021 May 31.

Thyroid, Head, and Neck Cancer (THANC) Foundation, 10 Union Square East, Suite 5B, New York, NY 10003, USA; Department of Otolaryngology-Head and Neck Surgery, Icahn School of Medicine at Mount Sinai, 10 Union Square East, Suite 5B, New York, NY 10003, USA.

Background: Primary malignancies arising in the external auditory canal (EAC) are rare and usually are treated surgically. We review techniques to reconstruct the EAC following ablative surgery, and introduce a rarely utilized tragal skin flap which has particular advantages for reconstruction of limited anterior EAC defects.

Methods: The terms "tragal flap", "external auditory canal", "preauricular tragal flap", "reconstructive techniques" were searched on PubMed and Google Scholar. Read More

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Beware of covert enemies: malignant otitis externa with base of the skull osteomyelitis, a case report and review of literature.

IDCases 2021 18;25:e01163. Epub 2021 May 18.

Department of Infectious Diseases, Communicable Diseases Centre, Hamad Medical Corporation, Qatar.

Background: Malignant otitis externa (MOE) is a serious infection of the external auditory canal that is frequently associated with skull base osteomyelitis (SBO) as well as secondary neurological sequelae. Patients with poorly controlled diabetes mellitus or immunosuppression are at increased risk of developing such critical infection for multiple local and systemic factors. While most cases are secondary to bacterial infections particularly, fungal infections are also occasionally encountered, often associated with delayed diagnosis and high morbidity and mortality. Read More

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Genus and Its Various Human Superficial and Cutaneous Features.

Pathogens 2021 May 23;10(6). Epub 2021 May 23.

Department of Hemobiology and Blood Transfusion, 'Hassani Abdelkader' Hospital, UDL Faculty of Medecine, Sidi-Bel-Abbes 22000, Algeria.

Superficial and cutaneous aspergillosis is a rare fungal disease that is restricted to the outer layers of the skin, nails, and the outer auditory canal, infrequently invading the deeper tissue and viscera, particularly in immunocompromised patients. These mycoses are acquired through two main routes: direct traumatic inoculation or inhalation of airborne fungal spores into paranasal sinuses and lungs. Lesions are classified into three categories: otomycosis, onychomycosis, and cutaneous aspergillosis. Read More

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Collaural fistula (Work Type II first branchial cleft anomaly) with prolonged morbidity: A case report.

SAGE Open Med Case Rep 2021 13;9:2050313X211014515. Epub 2021 May 13.

Department of Otolaryngology, Head and Neck Surgery, Graduate School of Medicine, Kyoto University, Kyoto, Japan.

Collaural fistula is a very rare Work Type II first branchial cleft anomaly in which there is a complete fistulous tract between external auditory canal and the neck. Misdiagnosis and mismanagement can lead to prolonged morbidity and complications due to repeated infections. We present a case of an 18-year-old lady with a recurrent discharging sinus on her neck for 4 years. Read More

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Rare case of ballooning herniation of the tympanic membrane.

BMJ Case Rep 2021 May 25;14(5). Epub 2021 May 25.

Department of Otorhinolaryngology, Head and Neck Surgery, Kantonsspital Aarau AG, Aarau, Aargau, Switzerland.

Herniation of the tympanic membrane is a rare benign malformation of the tympanic membrane into the external auditory canal. It may be asymptomatic or associated with symptoms such as aural fullness, tinnitus, otalgia or hearing loss. We present a case of a symptomatic herniation of the tympanic membrane and its surgical therapy with hernia excision and tympanoplasty. Read More

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Metallic foreign body adjacent to the round window: a rare cause for chronic tympanic membrane perforation with hearing loss.

BMJ Case Rep 2021 May 20;14(5). Epub 2021 May 20.

School of Medicine, The University of Western Australia, Perth, Western Australia, Australia.

Welding injuries to the tympanic membrane (TM) or middle ear are rare but can cause significant damage. These injuries occur when a hot spark or molten slag drops directly into the external auditory canal and through the action of gravity (because the ear is upright during such an injury), then risks burning through the TM. This can lead to a multitude of adverse consequences including chronic TM perforation, secondary purulent otorrhoea, facial palsy, taste disturbance, vertigo and hearing loss. Read More

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Reversible Hearing Impairment Due to Inferior Colliculi Compression by a Pineal Glial Cyst.

NMC Case Rep J 2021 Apr 2;8(1):79-84. Epub 2021 Apr 2.

Department of Neurosurgery, Graduate School of Biomedical Sciences, Tokushima University, Tokushima, Tokushima, Japan.

Pineal glial cysts associated with bilateral hearing impairment are very rare. Here, we present the case of a 13-year-old boy with a pineal cyst, which caused severe bilateral hearing impairment persisting from 6 years of age. When the patient was 6 years old, the bilateral hearing acuity was about 40 dB on audiometry. Read More

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Acute Psychosis in COVID-19: Is It Due to Favipiravir Treatment or Acute Viral Illness?

SN Compr Clin Med 2021 May 9:1-3. Epub 2021 May 9.

Department of Emergency Medicine, Mersin Toros State Hospital, Mersin, Turkey.

In this report, we present a case of acute psychosis that occurred after the loading dose of favipiravir, one of the most prescribed antiviral drugs in many countries, in a patient with COVID-19. A 31-year-old female was readmitted to the emergency department after 3 days of sleep disturbance, intermittent paranoid delusions, auditory hallucinations, and agitation that started after the favipiravir treatment was initiated. A physical examination revealed disorganized speech, distorted orientation, and agitation. Read More

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A case report of sudden-onset auditory neuropathy spectrum disorder associated with Brown-Vialetto-Van Laere syndrome (riboflavin transporter deficiency).

Int J Audiol 2021 May 13:1-7. Epub 2021 May 13.

Department of Neurology, Division of Child Neurology, Istanbul Faculty of Medicine, Istanbul University, Istanbul, Turkey.

Objective: The purpose of this paper is to describe a child with auditory neuropathy spectrum disorder (ANSD) associated with Brown-Vialetto-Van Laere (BVVL) syndrome, which is a rare, inherited, neurodegenerative disorder that is caused by defects in riboflavin transporter genes.

Design: We report the audiological and clinical profile of a child who presented with a complaint of sudden loss of speech understanding associated with an atypical form of ANSD. He was later diagnosed with BVVL. Read More

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Malignant Peripheral Nerve Sheath Tumor of the Cerebellar Hemisphere: An Unusual Location and Multiple Intracranial Parenchyma Metastases.

Cureus 2021 Apr 8;13(4):e14373. Epub 2021 Apr 8.

Department of Neurosurgery, Ningxia Key Laboratory of Cerebrocranial Disease, General Hospital of Ningxia Medical University, Yinchuan, CHN.

Malignant peripheral nerve sheath tumors (MPNSTs) are rare soft tissue malignancies that can occur in any part of the body. The most common sites are the proximal limbs and trunk. Intracranial MPNSTs are rare; most originate from the auditory, trigeminal, and other cranial nerves, and occurrence within the brain parenchyma is rarer. Read More

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Radiologic Findings in Pediatric Sudden Sensorineural Hearing Loss.

Otol Neurotol 2021 May 10. Epub 2021 May 10.

Department of Pediatrics, Children's Hospital of Atlanta, Atlanta, GA Department of Radiology and Imaging Sciences, Emory University Department of Radiology, Children's Healthcare of Atlanta, Atlanta, Georgia Department of Otolaryngology Head and Neck Surgery, University of Pennsylvania Children's Hospital of Philadelphia, Philadelphia, Pennsylvania.

Objective: Pediatric sudden sensorineural hearing loss (pSSNHL) is a rare phenomenon. There is currently no consensus on the role of imaging in the work-up for this patient population. We aim to evaluate the efficacy of magnetic resonance imaging (MRI) and high-resolution computed tomography (CT) studies in determining the etiology of pSSNHL. Read More

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A Large Intratemporal Facial Nerve Schwannoma Presenting as an Occluding External Auditory Canal Mass.

Ear Nose Throat J 2021 May 11:1455613211016706. Epub 2021 May 11.

Department of Otorhinolaryngology, 89681The Second Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou, People's Republic of China.

Facial nerve schwannomas are rare, benign, slow-growing tumors that can occur in any segment of the facial nerve, although 71% of cases are intratemporal. Surgical resection can lead to facial nerve injury. Facial function recovery after reanimation is usually not better than House-Brackmann (HB) grade III. Read More

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Intramedullary spinal cord metastasis from an adenoid cystic carcinoma of the external auditory canal: case report.

Br J Neurosurg 2021 May 10:1-5. Epub 2021 May 10.

Department of Neurosciences, Biomedicine and Movement Sciences, Section of Neurosurgery, Borgo Trento University Hospital, University of Verona, Verona, Italy.

Background And Importance: Intramedullary spinal cord metastases (ISCMs) are rare and no cases of ISCM from an adenoid cystic carcinoma (ACC) of the external auditory canal (EAC) have been reported.

Clinical Presentation: We report a 54-year old man complaining backpain and worsening myelopathy. He had an ACC of the EAC resected years prior. Read More

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The Effect of Learning English on P300 in Children.

Int Arch Otorhinolaryngol 2021 Apr 30;25(2):e284-e288. Epub 2020 Jun 30.

Post-graduate Program in Human Communication Disorders, Universidade Federal de Santa Maria (UFSM), Santa Maria, RS, Brazil.

 Learning a second language is an essential task in today's world, and is experienced by many children. The cognitive auditory-evoked potential (P300) is related to cognitive activity, attention and concentration, enabling the investigation of the effect of a second language on the central auditory pathway.  To analyze the effects of learning English on P300 latency and amplitude in children and to correlate them with age, time of exposure to English, and time in class. Read More

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Primary external auditory canal cholesteatoma of 301 ears: a single-center study.

Eur Arch Otorhinolaryngol 2021 May 4. Epub 2021 May 4.

Department of Otolaryngology, Ningde Municipal Hospital Affiliated of Ningde Normal College (Ningde Institute of Otolaryngology), Ningde, 352100, Fujian, China.

Purpose: Limited literature exists on primary external auditory canal (EAC) cholesteatoma (EACC). Here, we focus on the clinical features of this rare disease, especially the invasive patterns of lesion progression, through a large population study and present simple and practical staging.

Methods: In all, 276 patients (male 99; female 177; mean age 41. Read More

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Medial Acoustic Tumors: Special Considerations: 2-Dimensional Operative Video.

Oper Neurosurg (Hagerstown) 2021 Apr 30. Epub 2021 Apr 30.

Department of Neurosurgery, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts, USA.

Medial acoustic tumors are a rare distinct type of vestibular schwannoma having distinguished clinical and radiological features.1 Originating medially in the cerebellopontine angle without extending into the lateral internal auditory meatus, they are frequently giant in size at presentation in younger patients with a relatively preserved hearing, while they have other neurological deficits from cerebellar or brainstem compression and associated hydrocephalus. Imaging typically shows a cystic tumor with local mass effect and an internal auditory canal filled with cerebrospinal fluid. Read More

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Impaired Audiovisual Representation of Phonemes in Children with Developmental Language Disorder.

Brain Sci 2021 Apr 16;11(4). Epub 2021 Apr 16.

Department of Statistics, Purdue University, 250 N. University Street, West Lafayette, IN 47907-2066, USA.

We examined whether children with developmental language disorder (DLD) differed from their peers with typical development (TD) in the degree to which they encode information about a talker's mouth shape into long-term phonemic representations. Children watched a talker's face and listened to rare changes from [i] to [u] or the reverse. In the neutral condition, the talker's face had a closed mouth throughout. Read More

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A New Pathogenic Variant in Causing Deafness Due to an Incomplete Partition of the Cochlea Paved the Way for Innovative Surgery.

Genes (Basel) 2021 Apr 21;12(5). Epub 2021 Apr 21.

Department of Otorhinolaryngology, Head and Neck Surgery, Brussels Health Campus, Vrije Universiteit Brussel, 1090 Brussels, Belgium.

Incomplete partition type III (IP-III) is a relatively rare inner ear malformation that has been associated with a gene mutation. The IP-III anomaly is mainly characterized by incomplete separation of the modiolus of the cochlea from the internal auditory canal. We describe a 71-year-old woman with profound sensorineural hearing loss diagnosed with an IP-III of the cochlea that underwent cochlear implantation. Read More

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The role of sex chromosomes and sex hormones in vocal learning systems.

Horm Behav 2021 Jun 22;132:104978. Epub 2021 Apr 22.

Duke University Medical Center, The Rockefeller University, Howard Hughes Medical Institute, United States of America. Electronic address:

Vocal learning is the ability to imitate and modify sounds through auditory experience, a rare trait found in only a few lineages of mammals and birds. It is a critical component of human spoken language, allowing us to verbally transmit speech repertoires and knowledge across generations. In many vocal learning species, the vocal learning trait is sexually dimorphic, where it is either limited to males or present in both sexes to different degrees. Read More

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Acute Otitis Media and Facial Paralysis in an Infant with Aural Atresia: Management of a Rare Case.

J Int Adv Otol 2021 Mar;17(2):186-189

Departmenf of Otolaryngology, Erciyes University School of Medicine, Kayseri, Turkey.

Aural atresia is a congenital disease that is characterized by an embryologic developmental defect of the external auditory canal (EAC). There is an erythematous, bulging tympanic membrane by otoscope in physical examination of acute otitis media (AOM). Children with aural atresia experience AOM as children have normal anatomy. Read More

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Middle Ear Neuroendocrine Tumor: A Case Report and Review of the Literature in Pediatric Population.

J Int Adv Otol 2021 Mar;17(2):150-155

Department of Otorhinolaryngology, Head and Neck Surgery, Meyer Children's Hospital, Florence, Italy.

A rare pediatric case report of middle ear neuroendocrine tumor and review of the pediatric cases reported in the literature. A 16-year-old female showed a lesion occupying the posterosuperior part of the medial third of the right external auditory canal confirmed by computed tomography scan, without clear evidence of bone erosion. The patient underwent canal wall tympanoplasty in 1 stage. Read More

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Susac's syndrome - A new ocular finding and disease outcome.

Eye (Lond) 2021 Apr 20. Epub 2021 Apr 20.

Division of Ophthalmology, Tel Aviv Medical Center, Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.

Background: Susac's syndrome, a rare autoimmune vasculo-occlusive disease, may pose a diagnostic challenge and result in a devastating ocular and systemic outcome. Our study identifies a new retinal finding and evaluates disease outcome. We aimed to assess clinical and imaging findings, systemic manifestations and disease outcome in patients with ocular Susac's syndrome under immunosuppressive/immunomodulation therapies. Read More

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A Phenotypic Comparison of Loudness and Pain Hyperacusis: Symptoms, Comorbidity, and Associated Features in a Multinational Patient Registry.

Am J Audiol 2021 Apr 20:1-18. Epub 2021 Apr 20.

Department of Hearing and Speech Sciences, Vanderbilt University Medical Center, Nashville, TN.

Purpose Hyperacusis is a complex and poorly understood auditory disorder characterized by decreased tolerance to sound at levels that would not trouble most individuals. Recently, it has been suggested that individuals who experience otalgia in response to everyday sounds (termed ) may differ clinically from those whose primary symptom is the perception of everyday sounds as excessively loud (termed ). Despite this theoretical distinction, there have been no empirical studies directly comparing these two populations of hyperacusis patients. Read More

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Endoscopic Repair of a Temporal Bone Pneumatocele.

Otol Neurotol 2021 Apr 14. Epub 2021 Apr 14.

Kolling Deafness Research Centre, Royal North Shore Hospital, University of Sydney Department of Otolaryngology-Head and Neck Surgery, Faculty of Medicine and Health Sciences, Macquarie University, Sydney, Australia.

Objective: Pneumatoceles of the temporal bone are rare entities. A symptomatic external auditory canal pneumatocele repaired endoscopically is demonstrated.

Methods: A 79-year-old man presented with fluctuating hearing loss and difficulty wearing in-ear hearing aids. Read More

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Inner ear salivary gland choristoma extending to the middle ear with congenital profound hearing loss and facial palsy: a case report.

J Otolaryngol Head Neck Surg 2021 Apr 15;50(1):25. Epub 2021 Apr 15.

Department of Otolaryngology-Head and Neck Surgery, Keio University School of Medicine, 35 Shinanomachi, Shinjuku-ku, Tokyo, 160-8582, Japan.

Background: Salivary gland choristoma (SGCh) is a rare benign tumor reported in several unusual sites, such as the gastrointestinal tract, the optic nerve, and the internal auditory canal, but never reported in the inner ear.

Case Presentation: An 8-year-old girl with a history of left profound congenital hearing loss presented to us with ipsilateral progressive severe facial nerve palsy (House-Brackmann Grade VI). The left tympanic membrane was swollen with a pulsatile tumor. Read More

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Case Report: Could Hennebert's Sign Be Evoked Despite Global Vestibular Impairment on Video Head Impulse Test? Considerations Upon Pathomechanisms Underlying Pressure-Induced Nystagmus due to Labyrinthine Fistula.

Front Neurol 2021 29;12:634782. Epub 2021 Mar 29.

ENT Unit, Department of Surgery, Azienda USL-IRCCS di Reggio Emilia, Reggio Emilia, Italy.

We describe a case series of labyrinthine fistula, characterized by Hennebert's sign (HS) elicited by tragal compression despite global hypofunction of semicircular canals (SCs) on a video-head impulse test (vHIT), and review the relevant literature. All three patients presented with different amounts of cochleo-vestibular loss, consistent with labyrinthitis likely induced by labyrinthine fistula due to different temporal bone pathologies (squamous cell carcinoma involving the external auditory canal in one case and middle ear cholesteatoma in two cases). Despite global hypofunction on vHIT proving impaired function for each SC for high accelerations, all patients developed pressure-induced nystagmus, presumably through spared and/or recovered activity for low-velocity canal afferents. Read More

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Ceruminous adenocarcinoma of the external auditory canal- report of a rare case with a discussion of its differential diagnoses.

Indian J Pathol Microbiol 2021 Apr-Jun;64(2):424-426

Department of Pathology, All India Institute of Medical Sciences, Raipur, India.

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Assessing the integrity of auditory processing and sensory memory in adults with cystinosis (CTNS gene mutations).

Orphanet J Rare Dis 2021 Apr 13;16(1):177. Epub 2021 Apr 13.

Department of Pediatrics, Albert Einstein College of Medicine, Van Etten Building, Suite 1C, 1225 Morris Park Avenue, Bronx, NY, 10461, USA.

Background: Cystinosis, a rare lysosomal storage disease, is characterized by cystine crystallization and accumulation within tissues and organs, including the kidneys and brain. Its impact on neural function appears mild relative to its effects on other organs, but therapeutic advances have led to substantially increased life expectancy, necessitating deeper understanding of its impact on neurocognitive function in adulthood. We previously demonstrated intact auditory sensory processing, accompanied by mild sensory memory difficulties, in children and adolescents with cystinosis. Read More

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