Hearing Loss

Delayed recovery of speech discrimination after fractionated stereotactic radiotherapy for vestibular schwannoma in neurofibromatosis 2

February 12, 2014     Michael Hoa, MD; Eric P. Wilkinson, MD; and William H. Slattery III, MD
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Abstract

Hearing loss commonly occurs after radiation therapy for an acoustic neuroma, and it is highly unusual for hearing to return after a prolonged period of time. We report the case of a 12-year-old boy with neurofibromatosis 2 who underwent fractionated stereotactic radiotherapy for the treatment of a left-sided vestibular schwannoma. Following treatment, he demonstrated an elevation of pure-tone audiometric thresholds and a sudden decrease in speech discrimination score (SDS) to 0%. However, 20 months postoperatively, his SDS suddenly and spontaneously rose to 92%, although there was no improvement in his speech reception threshold. We discuss the possible reasons for the unusual outcome in this patient.

Temporal bone fracture

January 21, 2014     Danielle M. Blake, BA; Senja Tomovic, MD; Robert W. Jyung, MD
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Transverse fractures account for approximately 20% of temporal bone fractures. They occur secondary to frontal or occipital head trauma, and they run perpendicular to the petrous pyramid.

Bilateral middle cranial fossa encephaloceles presenting as conductive hearing loss

December 20, 2013     Colleen T. Plein, MD; Alexander J. Langerman, MD; Miriam I. Redleaf, MD
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Abstract

We report a case involving a patient with bilateral middle cranial fossa encephaloceles extending into the middle ear and causing conductive hearing loss. An obese, 47-year-old woman with a history of a seizure disorder presented with a slow-onset subjective hearing loss. Examination revealed opaque tympanic membranes, and audiometry showed a mixed hearing loss bilaterally. Myringotomy demonstrated soft tissue behind each tympanic membrane. Biopsy, computed tomography, magnetic resonance imaging, and mastoidectomy confirmed the diagnosis of bilateral middle cranial fossa encephaloceles. Bilateral encephaloceles are uncommon, and the resulting bilateral conductive hearing loss secondary to mechanical obstruction of ossicular vibration is even more rare. This patient's obesity and seizures perhaps contributed to her disease process.

Vertebral artery dissection: An unusual cause of transient ataxia, vertigo, and sensorineural hearing loss

December 20, 2013     Leila L. Touil, MBChB; Glen James Watson, FRCS, DOHNS; Michael Small, FRCS
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Abstract

We present the case of a 33-year-old man who was admitted with intermittent ataxia, vertigo, and sensorineural hearing loss as a result of a vertebral artery dissection following minor neck trauma. Our aim is to highlight the importance of obtaining magnetic resonance imaging, magnetic resonance angiography, and/or duplex color-flow imaging when presented with a case of fluctuating vertigo and sensorineural hearing loss with side-specific ataxia. Likewise, it is important to obtain the input of neurologists to optimize a patient's prognosis and minimize long-term sequelae.

Extrusion of hydroxyapatite ossicular prosthesis

October 23, 2013     Danielle M. Blake, BA; Senja Tomovic, MD; Robert W. Jyung, MD
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Extrusion of hydroxyapatite prostheses is unfortunately a common complication of middle ear surgery.

Paraganglioma presenting as cholesterol granuloma of the petrous apex

September 18, 2013     Selena E. Heman-Ackah, MD, MBA; Tina C. Huang, MD
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Abstract

We report the unique finding of a petrous apex cholesterol granuloma associated with a paraganglioma, also known as a glomus jugulare tumor, in a 52-year-old woman who presented to our department with pulsatile tinnitus, hearing loss, aural fullness, and disequilibrium. She had been treated for a petrous apex cholesterol granuloma 20 years earlier, at which time she had undergone drainage of the granuloma via subtotal petrous apicectomy. When she came to our facility approximately 20 years later, she had signs and symptoms consistent with a jugular paraganglioma, which was likely to have been present at the time of her initial presentation for the cholesterol granuloma. In fact, microscopic bleeding from the paraganglioma might have led to the formation of the cholesterol granuloma. The metachronous presentation of these two entities, which to our knowledge has not been reported previously in the literature, indicates the potential association of paragangliomas with the formation of cholesterol granulomas of the petrous apex.

Medial canal fibrosis

September 18, 2013     Joseph A. Ursick, MD; John W. House, MD
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Medial canal fibrosis is an uncommon condition characterized by progressive stenosis of the bony external auditory canal.

Transient auditory dysfunction: A description and study of prevalence

August 21, 2013     Laurence Maximilian Almond, MB ChB; Ketul Patel, MB ChB; and Darius Rejali, MB ChB
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Abstract

Transient auditory dysfunction (TAD) is a previously undescribed symptom complex of unknown cause. It is characterized by short-lasting sensorineural hearing loss (unilateral or bilateral), it is associated with tinnitus, it resolves completely within minutes, and it is not accompanied by vestibular symptoms. We conducted a cross-sectional prospective study to define TAD, find its prevalence, and discuss its significance. Two hundred healthy subjects between the ages of 16 and 49 years were surveyed using a questionnaire. Of these subjects, 41 (20.5%) reported experiencing symptoms of TAD. The mean number of episodes was 5.9 times per month, the mean duration was 41 seconds, and 80% experienced concomitant tinnitus. We conclude that TAD is a common finding in a healthy population. This may have implications for the pathogenesis of sudden-onset sensorineural hearing loss. Further longitudinal studies and detailed audiologic evaluation of patients with TAD are required to ascertain the significance, etiology, and pathophysiology of this condition.

Cicatricial external auditory canal stenosis caused by ectodermal dysplasia: Rapp-Hodgkin syndrome

June 11, 2013     Amanda B. Sosulski, MD and James D. Hayes, MD
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Abstract

We present a case of recurrent cicatricial stenosis of the external ear canals caused by ectodermal dysplasia, specifically Rapp-Hodgkin syndrome, in a 45-year-old woman. No form of medical or surgical management has produced durable patency of the patient's ear canals, and her hearing loss is being managed with hearing aids. Topical management of the recurring external otitis slows the process but has been unsuccessful in preventing restenosis of both external auditory canals.

Behcet disease as a cause of hearing loss: A prospective, placebo-controlled study of 29 patients

March 24, 2013     Ozgur Kemal, MD; Yucel Anadolu, MD; Ayse Boyvat, MD; and Ahmet Tataragasi, AuD
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Abstract

We conducted a prospective, placebo-controlled study to determine the incidence and severity of inner ear involvement and hearing loss in patients with Behçet disease. Our study population was made up of 29 patients with Behçet disease and 28 healthy controls. Audiometric pure-tone thresholds and transient evoked otoacoustic emission (TEOAE) levels were determined in both groups. The main outcome measures were pure-tone audiometry (PTA) levels and TEOAE levels in the two groups. PTA detected a sensorineural hearing loss in 10 of the 29 patients (34.5%). The difference in audiometric findings between the two groups was statistically significant at 1, 2, 4, and 8 kHz (p ≤ 0.0498). A comparison of TEOAE levels revealed that the difference in sound-to-noise ratio between the two groups was not significant at 1, 1.5, 2, and 3 kHz, but it was significant in 4 kHz (p = 0.02), and the difference in reproducibility between the two groups was significant at 2 and 4 kHz (p ≤ 0.03). We conclude that all patients with Behçet disease should be screened for hearing impairment and subsequently treated if an impairment is discovered.

An extruded ossicular prosthesis

February 25, 2013     Willis S.S. Tsang, FRCSEd(ORL); Michael C.F. Tong, MD(CUHK); C. Andrew van Hasselt, M Med(Otol)
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Extrusion of ossicular prostheses results from postoperative tympanic membrane medialization. The incorporation of cartilage grafting material is recommended to minimize extrusion.

Primary mucosal melanoma arising from the eustachian tube with CTLA-4, IL-17A, IL-17C, and IL-17E upregulation

January 24, 2013     Calvin Wei, MD; Sasis Sirikanjanapong, MD; Seth Lieberman, MD; Mark Delacure, MD; Frank Martiniuk, PhD; William Levis, MD; Beverly Y. Wang, MD
article

Abstract

Primary malignant melanoma arising from the eustachian tube is extremely rare. We report the case of a 63-year-old white man who presented with a 1-month history of left-sided hearing loss and aural fullness. Flexible fiberoptic laryngoscopy detected a blue-purple mass that appeared to arise from the left lateral nasopharynx. Computed tomography demonstrated an enhancing mass arising from an orifice of the left eustachian tube. The tumor was debulked endoscopically and was confirmed to have originated in the left eustachian tube. Histologically, the tumor was made up of heavily pigmented pleomorphic spindle cells with frequent mitoses. The tumor cells were immunohistochemically positive for S-100 protein, HMB-45, Melan-A, and PNL-2. The final diagnosis was a mucosal malignant melanoma. We also performed a nested polymerase chain reaction assay for several genes of interest, including CTLA-4, IL-17A, IL-17B, IL-17C, IL-17D, IL-17E, IL-17F, PLZF, Foxp3, RORγt, CD27, and CD70. These genes have been studied mainly in cutaneous melanomas, especially for the development of immunotherapy, but only very limited studies have been done on mucosal melanomas. Our investigation found upregulation of CTLA-4, IL-17A, IL-17C, and IL-17E. Based on our finding of CTLA-4 upregulation, it may be suggested that our patient might have had low antitumor immunity and that he might have benefited from CTLA-4 blockade. On the other hand, upregulation of IL-17A and IL-17E might reflect increased antitumor immunity, which could suggest that patients with a mucosal melanoma might benefit from immunomodulators associated with the effect of Th17. These genes also have great potential to help melanoma patients obtain tailored treatment, and they can be used as biomarkers for predicting prognosis.

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