RESUMEN
PURPOSE OF REVIEW: This article reviews the causes of tinnitus, hyperacusis, and otalgia, as well as hearing loss relevant for clinicians in the field of neurology. RECENT FINDINGS: Important causes of unilateral and bilateral tinnitus are discussed, including those that are treatable or caused by serious structural or vascular causes. Concepts of hyperacusis and misophonia are covered, along with various types of neurologic disorders that can lead to pain in the ear. Hearing loss is common but not always purely otologic. SUMMARY: Tinnitus and hearing loss are common symptoms that are sometimes related to a primary neurologic disorder. This review, tailored to neurologists who care for patients who may be referred to or encountered in neurology practice, provides information on hearing disorders, how to recognize when a neurologic process may be involved, and when to refer to otolaryngology or other specialists.
Asunto(s)
Pérdida Auditiva , Acúfeno , Dolor de Oído/diagnóstico , Dolor de Oído/etiología , Pérdida Auditiva/diagnóstico , Humanos , Hiperacusia/diagnóstico , Acúfeno/diagnóstico , Acúfeno/etiología , Acúfeno/terapiaRESUMEN
BACKGROUND: Antiglutamic acid decarboxylase (GAD)-associated neurologic disorders are rare, with varied presentations, including stiff-person syndrome (SPS) and cerebellar ataxia (CA). Vestibular and ocular motor (VOM) dysfunction can be the main presentation in a subset of patients. METHODS: Retrospective review of the Johns Hopkins Hospital medical records from 1997 to 2018 identified a total of 22 patients with a diagnosis of anti-GAD-associated SPS or CA who had detailed VOM assessments. Eight had prominent VOM dysfunction at the initial symptom onset and were referred to neurology from ophthalmology or otolaryngology ("early dominant"). Fourteen patients had VOM dysfunction that was not their dominant presentation and were referred later in their disease course from neurology to neuro-ophthalmology ("nondominant"). We reviewed clinical history, immunological profiles, and VOM findings, including available video-oculography. RESULTS: In the 8 patients with early dominant VOM dysfunction, the average age of symptom onset was 53 years, and 5 were men. The most common symptom was dizziness, followed by diplopia. Seven had features of CA, and 4 had additional features of SPS. None had a structural lesion on brain MRI accounting for their symptoms. The most common VOM abnormalities were downbeating and gaze-evoked nystagmus and saccadic pursuit. All received immune therapy and most received symptomatic therapy. Most experienced improvement in clinical outcome measures (modified Rankin scale and/or timed 25-foot walk test) or VOM function. By contrast, in the 14 patients in whom VOM dysfunction was nondominant, most had an SPS phenotype and were women. VOM abnormalities, when present, were more subtle, although mostly still consistent with cerebellar and/or brainstem dysfunction. CONCLUSIONS: Individuals with anti-GAD-associated neurologic disorders may present with prominent VOM abnormalities at the initial symptom onset that localize to the cerebellum and/or brainstem. In our cohort, immune and symptomatic therapies improved clinical outcomes and symptomatology.