Hearing Loss

HLA-B27-associated bilateral Ménière disease

March 1, 2010     Shamila G. Rawal, MD, Kunal H. Thakkar, MD, Kasra Ziai, BS, Peter A. Santi, MD, and Hamid R. Djalilian, MD
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Abstract

We describe 2 cases of bilateral Ménière disease with features resembling autoimmune inner ear disease in patients who were found to be carriers of human leukocyte antigen (HLA) B27. For immunohistochemical analysis, mouse inner ear sections were used as the tissue substrate for reaction with serum. Both patients demonstrated an increased immunofluorescence reaction compared with a normal control. We suggest that an antibody-mediated mechanism may be responsible for HLA-B27-associated Ménière disease.

Cerebellopontine angle ganglionic hamartoma: Case report

March 1, 2010     Sidrah M. Ahmad, BS, Fernando Garcia, MD, and Robert T. Sataloff, MD, DMA, FACS
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Abstract

Cerebellopontine angle (CPA) ganglionic hamartomas are rare. We present a case of a 49-year-old woman who presented with left-sided hearing loss of more than 4 years' duration and vertigo since childhood. Magnetic resonance imaging revealed a mass on the intracanalicular segment of cranial nerve VIII on the left side, consistent with acoustic schwannoma. She opted for observation of the tumor. The patient returned 21 months after the initial evaluation with new-onset left facial weakness. At this time, a working diagnosis of facial nerve neuroma was made. The lesion was excised, and pathology confirmed a diagnosis of left-sided CPA ganglionic hamartoma. Otolaryngologists should be familiar with this uncommon tumor and include it in the differential diagnosis of CPA lesions.

High jugular bulb

February 1, 2010     S. Arif Ulubil, MD and Murat Topdag, MD
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“Gun-shooting hearing loss”: A pilot study

January 1, 2010     Joseph Sataloff, MD, DSc, Mary J. Hawkshaw, BSN, RN, CORLN, and Robert T. Sataloff, MD, DMA, FACS
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Abstract

Gun-shooting deafness is the common terminology applied to sensorineural hearing loss caused by shooting firearms. Many characteristics of gun-shooting hearing loss have been proposed, but they have not been defined clearly or established conclusively. We studied 37 users of recreational firearms to obtain pilot data to help determine if it is true that right-handed gun shooters develop more hearing loss in the left ear and vice versa, whether everyone who frequently shoots guns develops sensorineural hearing loss, and whether significant hearing loss is typically prevented by wearing commercially available ear protectors while shooting.

Clinical significance of a hearing and imaging workup in a child with cochlear nerve aplasia and a normal internal auditory canal

December 1, 2009     Fernando Ribeiro, MD, Liciere Marotta, MD, and Claudia A. Eckley, MD
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Abstract

Modern magnetic resonance imaging has significantly improved the diagnosis of cochlear nerve deficiencies. A careful assessment of all clinical, imaging, and auditory data is of utmost importance in such cases in order to properly establish the site of the abnormality. We report the case of a 3-year-old girl with unilateral cochlear nerve aplasia, normal middle and inner ear anatomy, and an absence of otoacoustic emissions, all of which erroneously suggested cochlear damage. We also briefly review the embryogenesis of the inner ear and auditory pathway. A patient with similar findings in the setting of a bilateral hearing loss and insufficient imaging would be at risk of inappropriate cochlear implantation.

Epidemiologic study to explore links between Ménière syndrome and migraine headache

October 31, 2009     Quinton Gopen, MD, Erik Viirre, MD, PhD, and John Anderson, PhD
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Abstract

Many authors have noted an association between Ménière syndrome and migraine headache. In an attempt to explore a possible link between these two disorders, we performed an epidemiologic study. The National Health Interview Survey (NHIS) includes interviews with tens of thousands of patients annually to estimate the incidence of various diseases. In this study the data collected from the NHIS for the years 1986 to 1988 and 1994 were analyzed to determine the incidence of Ménière syndrome and migraine headache. A total of 423,400 individuals were interviewed over the combined 4 years included in this evaluation. The incidence of migraine headache was calculated at 3.8%, and the incidence of Ménière syndrome was estimated at 0.14%. The incidence of migraine headache in patients with Ménière syndrome was estimated at 4.5%. The incidence of migraine headache was not substantially elevated in patients with Ménière syndrome when compared to the general population.

Correlation of central auditory processing deficits and vascular loop syndrome

September 30, 2009     Jennifer B. Shinn, PhD, Matthew L. Bush, MD, and Raleigh O. Jones, MD
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Abstract

We report a case involving a 49-year-old woman with vascular loop syndrome. The patient was evaluated because of complaints of decreased hearing sensitivity in her right ear. Central auditory tests were performed. The patient was found to have an asymmetry on pure-tone audiometry and an auditory processing deficit, suggesting central pathology. Magnetic resonance imaging confirmed central involvement, revealing a vascular loop extending into the right internal auditory canal. This case report demonstrates that auditory deficits may result from vascular loop compression and that these deficits may benefit from a battery of tests to help identify and localize the pathology.

Applebaum incudostapedial prosthesis

August 31, 2009     James Lin, MD and Jose N. Fayad, MD
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Complete round window niche occlusion for superior semicircular canal dehiscence syndrome: A minimally invasive approach

July 31, 2009     Herbert Silverstein, MD, FACS and Mark J. Van Ess, DO
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Abstract

Superior semicircular canal dehiscence (SCD) syndrome is a recognized condition associated with varying degrees of vestibular and auditory dysfunction. The authors present a case study of disabling SCD syndrome in a 37-year-old man who was successfully treated with a complete round window niche occlusion via a transcanal approach. This case represents the first report of a transcanal complete round window niche occlusion for the treatment of SCD syndrome. A brief literature review and discussion of surgical techniques follow.

Synovial chondromatosis of the middle ear: A case report

June 30, 2009     Domenico Saggese, MD, Ignacio Javier Fernandez, MD, Nunzio C.M. Salfi, MD, and Alberto Rinaldi Ceroni, MD
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Abstract

Synovial chondromatosis is a rare pathology of unknown etiology characterized by multinodular cartilaginous proliferation in the synovium. When it does occur, it usually develops in the large synovial joints (e.g., the knee, hip, elbow, and shoulder). Only rarely does it occur in the head and neck (most often in the temporomandibular joint). We report a case of synovial chondromatosis of the middle ear (i.e., the incudomalleolar joint) in a 64-year-old woman, and we describe the clinical, radiologic, and intraoperative findings in this case. To the best of our knowledge, this is the first reported case of synovial chondromatosis in the middle ear.

Ear trauma caused by a yucca plant leaf spine

May 31, 2009     Yoav P. Talmi, MD, FACS, Michael Wolf, MD, Lela Migirov, MD, and Jona Kronenberg, MD
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Abstract

Three uncommon cases of ear trauma caused by a yucca plant leaf spine are presented. One patient presented with tympanic perforation and the second with mixed hearing loss after spontaneous closure. The third patient probably had a perilymphatic fistula with subsequent labyrinthitis and hearing loss. Although the yucca is a ubiquitous plant, to the best of our knowledge, such incidents have not been previously reported.

Dizziness in a SCUBA diver

April 30, 2009     Kenneth H. Brookler, MD, MS, FRCSC
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