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2023, Number 3

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Otorrinolaringología 2023; 68 (3)

Sudden hearing loss as an initial manifestation of Arnold-Chiari type I malformation

Ochoa CMF, Camargo VN, Montes OEJ, Ponce PJM, Cisneros LJC
Full text How to cite this article

Language: Spanish
References: 13
Page: 139-146
PDF size: 206.18 Kb.


Key words:

Hearing loss, sudden, Hearing loss, sensorineural, Arnold-Chiari malformation, Magnetic resonance spectroscopy.

ABSTRACT

Background: A sudden hearing loss is defined as an auditory loss greater than 30 decibels over at least three contiguous frequencies, within a 72-hour period. It has different aetiologies, although it is often considered idiopathic. The viral aetiology (caused by viruses of the Herpesviridae family) and the vascular are the most common. As part of the diagnostic process, a magnetic resonance is performed in all cases of sudden hearing loss to rule out retrocochlear disease. Currently, the use of systemic or intratympanic steroids during the first three weeks of onset is the basis of treatment.
Clinical case: A 57-year-old female patient with left ear sudden hearing loss with associated tinnitus, vertigo, and dizziness. On physical examination, she was found to have unilateral sensorineural hearing loss, determined by pure-tone audiometry, as well as a vertical, down-beating nystagmus observed during otoneurologic exploration. Magnetic resonance showed a significant herniation of the cerebellum tonsils through the foramen magnum (compatible with an Arnold-Chiari type I malformation). Systemic and intratympanic steroids were prescribed without success, so management aimed at rehabilitation and close monitoring.
Conclusions: Sudden hearing loss secondary to an Arnold-Chiari type 1 malformation is infrequent, especially as an isolated or initial symptom. The inferior extension of the magnetic resonance performed as part of the diagnostic algorithm of sudden hearing loss, to evaluate the cerebellum tonsils, may be an effective measure to rule out this condition in such patients.


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Otorrinolaringología. 2023;68