PURPOSE: There is evidence of progressive antibody-mediated loss of acetylcholine receptors on outer hair cells in the inner ear that underlies hearing dysfunction in myasthenia gravis patients. In this paper, we present the case of a 35-year old patient with adult-onset myasthenia gravis and thymoma who experienced progressive hearing loss and bilateral high-pitched tone tinnitus worsening over time. MATERIALS AND METHODS: Patient underwent Pure Tone audiometry, Acoustic Immittance test, Decay test, Transient-Evoked and Distortion Product Otoacustic Emissions with and without Contralateral Acoustic Stimulation, somatosensory tinnitus screening maneuvers, and self-assessment questionnaires for tinnitus, hearing loss and hyperacusis, in addition to anamnestic interview and complete ENT visit. Questionnaires for psychiatric comorbidities (SCL90R - Symptom Checklist 90 Revisited) were also administered to the patient. The patient was studied in baseline condition and 1h after administration of the acetylcholinesterase inhibitor pyridostigmine bromide, a drug that has been reported to have a temporary effect on outer hair cell electromotility in myasthenia gravis patients. RESULTS: Pure-tone audiometry showed a progressive hearing loss for high frequencies. Consistently, Transient-evoked and Distortion Product Otoacoustic Emission amplitudes were significantly reduced for mid and, especially, high frequencies; after drug administration, a significant otoacoustic emission amplitude increase was recorded. CONCLUSION: Our findings documented a chronic dysfunction of outer hair cells consistent with acetylcholine receptor loss; these alterations could represent a basis for development of hearing loss and tinnitus.
Development of progressive hearing loss and tinnitus in a patient with myasthenia gravis: an overlooked comorbidity? / Ralli, Massimo; DI STADIO, Arianna; Greco, Antonio; Altissimi, Giancarlo; Mazzei, Filippo; Turchetta, Rosaria; DE VINCENTIIS, Marco; Cianfrone, Giancarlo. - In: HEARING, BALANCE AND COMMUNICATION. - ISSN 2169-5717. - 15:4(2017), pp. 260-266. [10.1080/21695717.2017.1380968]
Development of progressive hearing loss and tinnitus in a patient with myasthenia gravis: an overlooked comorbidity?
RALLI, MASSIMO
Primo
;DI STADIO, AriannaSecondo
;GRECO, Antonio;ALTISSIMI, Giancarlo;MAZZEI, FILIPPO;TURCHETTA, Rosaria;DE VINCENTIIS, MarcoPenultimo
;CIANFRONE, GiancarloUltimo
2017
Abstract
PURPOSE: There is evidence of progressive antibody-mediated loss of acetylcholine receptors on outer hair cells in the inner ear that underlies hearing dysfunction in myasthenia gravis patients. In this paper, we present the case of a 35-year old patient with adult-onset myasthenia gravis and thymoma who experienced progressive hearing loss and bilateral high-pitched tone tinnitus worsening over time. MATERIALS AND METHODS: Patient underwent Pure Tone audiometry, Acoustic Immittance test, Decay test, Transient-Evoked and Distortion Product Otoacustic Emissions with and without Contralateral Acoustic Stimulation, somatosensory tinnitus screening maneuvers, and self-assessment questionnaires for tinnitus, hearing loss and hyperacusis, in addition to anamnestic interview and complete ENT visit. Questionnaires for psychiatric comorbidities (SCL90R - Symptom Checklist 90 Revisited) were also administered to the patient. The patient was studied in baseline condition and 1h after administration of the acetylcholinesterase inhibitor pyridostigmine bromide, a drug that has been reported to have a temporary effect on outer hair cell electromotility in myasthenia gravis patients. RESULTS: Pure-tone audiometry showed a progressive hearing loss for high frequencies. Consistently, Transient-evoked and Distortion Product Otoacoustic Emission amplitudes were significantly reduced for mid and, especially, high frequencies; after drug administration, a significant otoacoustic emission amplitude increase was recorded. CONCLUSION: Our findings documented a chronic dysfunction of outer hair cells consistent with acetylcholine receptor loss; these alterations could represent a basis for development of hearing loss and tinnitus.File | Dimensione | Formato | |
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