Superior semicircular canal dehiscence simulating otosclerosis. 2003

G Michael Halmagyi, and Swee T Aw, and Leigh A McGarvie, and Michael J Todd, and Andrew Bradshaw, and Robyn A Yavor, and Paul A Fagan
Neurology Department, Royal Prince Alfred Hospital, Sydney, and the Otolaryngology Department, St Vincent's Hospital, Sydney, Australia. Michael@icn.usyd.edu.au

This is a report of a patient with an air-bone gap, thought 10 years ago to be a conductive hearing loss due to otosclerosis and treated with a stapedectomy. It now transpires that the patient actually had a conductive hearing gain due to superior semicircular canal dehiscence. In retrospect for as long as he could remember the patient had experienced cochlear hypersensitivity to bone-conducted sounds so that he could hear his own heart beat and joints move, as well as a tuning fork placed at his ankle. He also had vestibular hypersensitivity to air-conducted sounds with sound-induced eye movements (Tullio phenomenon), pressure-induced nystagmus and low-threshold, high-amplitude vestibular-evoked myogenic potentials. Furthermore some of his acoustic reflexes were preserved even after stapedectomy and two revisions. This case shows that if acoustic reflexes are preserved in a patient with an air-bone gap then the patient needs to be checked for sound- and pressure-induced nystagmus and needs to have vestibular-evoked myogenic potential testing. If there is sound- or pressure-induced nystagmus and if the vestibular-evoked myogenic potentials are also preserved, the problem is most likely in the floor of the middle fossa and not in the middle ear, and the patient needs a high-resolution spiral computed tomography (CT) of the temporal bones to show this.

UI MeSH Term Description Entries
D007759 Labyrinth Diseases Pathological processes of the inner ear (LABYRINTH) which contains the essential apparatus of hearing (COCHLEA) and balance (SEMICIRCULAR CANALS). Inner Ear Disease,Ear Disease, Inner,Ear Diseases, Inner,Inner Ear Diseases,Labyrinth Disease
D008297 Male Males
D010040 Otosclerosis Formation of spongy bone in the labyrinth capsule which can progress toward the STAPES (stapedial fixation) or anteriorly toward the COCHLEA leading to conductive, sensorineural, or mixed HEARING LOSS. Several genes are associated with familial otosclerosis with varied clinical signs. Otospongiosis,Otoscleroses,Otospongioses
D012027 Reflex, Vestibulo-Ocular A reflex wherein impulses are conveyed from the cupulas of the SEMICIRCULAR CANALS and from the OTOLITHIC MEMBRANE of the SACCULE AND UTRICLE via the VESTIBULAR NUCLEI of the BRAIN STEM and the median longitudinal fasciculus to the OCULOMOTOR NERVE nuclei. It functions to maintain a stable retinal image during head rotation by generating appropriate compensatory EYE MOVEMENTS. Vestibulo-Ocular Reflex,Reflex, Vestibuloocular,Reflexes, Vestibo-Ocular,Reflexes, Vestibuloocular,Reflex, Vestibulo Ocular,Reflexes, Vestibo Ocular,Vestibo-Ocular Reflexes,Vestibulo Ocular Reflex,Vestibuloocular Reflex,Vestibuloocular Reflexes
D001844 Bone Conduction Transmission of sound waves through vibration of bones in the SKULL to the inner ear (COCHLEA). By using bone conduction stimulation and by bypassing any OUTER EAR or MIDDLE EAR abnormalities, hearing thresholds of the cochlea can be determined. Bone conduction hearing differs from normal hearing which is based on air conduction stimulation via the EAR CANAL and the TYMPANIC MEMBRANE. Bone Conduction Hearing,Conduction Hearing, Bone,Conduction, Bone,Hearing, Bone Conduction
D003937 Diagnosis, Differential Determination of which one of two or more diseases or conditions a patient is suffering from by systematically comparing and contrasting results of diagnostic measures. Diagnoses, Differential,Differential Diagnoses,Differential Diagnosis
D005072 Evoked Potentials, Auditory The electric response evoked in the CEREBRAL CORTEX by ACOUSTIC STIMULATION or stimulation of the AUDITORY PATHWAYS. Auditory Evoked Potentials,Auditory Evoked Response,Auditory Evoked Potential,Auditory Evoked Responses,Evoked Potential, Auditory,Evoked Response, Auditory,Evoked Responses, Auditory,Potentials, Auditory Evoked
D006311 Hearing Disorders Conditions that impair the transmission of auditory impulses and information from the level of the ear to the temporal cortices, including the sensorineural pathways. Distorted Hearing,Dysacusis,Paracousis,Paracusis,Hearing Disorder,Hearing, Distorted
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000368 Aged A person 65 years of age or older. For a person older than 79 years, AGED, 80 AND OVER is available. Elderly

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