Rie Naito

Questionnaire and interview in screening for hearing impairment in adults

Conclusion. Questionnaire and brief interview played an important complementary role in the mass hearing screening. Objectives. To evaluate the efficacy of mass screening for hearing loss. Subjects and methods. Review of a 9-year prospective screening (n=31 902) in a university. The screening comprised pure tone hearing screening at two frequencies (1000 Hz and 4000 Hz) and a questionnaire. Brief interview was introduced during the later 4-year period. The final diagnosis was made in the university hospital. Results. In hearing screening tests, approximate sensitivity was 89% for 1000 Hz and 91% for 4000 Hz. Approximate specificity was 89% and 88% for 1000 Hz and 4000 Hz, respectively. Brief interview with the subjects on the spot improved the accuracy, especially in specificity. Low tone sensorineural hearing loss, exudative otitis media and chronic otitis media could be overlooked without questionnaires.

Laryngeal dystonia in a case of severe motor and intellectual disabilities due to Japanese encephalitis sequelae

Laryngeal dystonia is characterized by stridor due to vocal cord dystonia and is observed in extrapyramidal disorders. Recently, botulinum toxin injection has been used as a primary therapy. Generally, severe motor and intellectual disabilities (SMID) are frequently complicated by various types of respiratory disorders. We report a SMID case with Japanese encephalitis sequelae showing repeated vocal cord abductor disturbance due to laryngeal dystonia, in addition to generalized dystonia, in whom MRI revealed basal ganglia lesions. Tracheostomy was effective for the case, and we believe that botulinum toxin injection may be inappropriate in SMID, both ethically and technically. Also, laryngeal dystonia should be considered as a cause of respiratory disorders in SMID.

Hypertrophic cranial pachymeningitis presenting as steroid-responsive hearing loss

Hypertrophic cranial pachymeningitis (HCP) is a chronic and progressive inflammatory disease that usually results in diffuse thickening of the dura mater. It often causes headache and multiple cranial nerve palsies due to fibrous entrapment or ischemic damage.1,2 Although several causes, either infectious or autoimmune, have been suggested, most aspects of this rare disease remain to be elucidated. It is usually diagnosed by contrast-enhanced magnetic resonance imaging (MRI) or histologic examination of the excised dura. Corticosteroid therapy has been effective in alleviating symptoms and arresting the progression of HCP.3

Auditory Brainstem Response and Neuropathology in a Case of Systemic T-Cell Lymphoma with Intracranial Metastasis

A 58-year-old male with systemic T-cell lymphoma involving the brainstem eccentrically, showed varying abnormal auditory brainstem responses (ABRs). The initial ABR demonstrated a prolonged V wave peak latency and an I-V IPL on admission. Four months later, all waves were absent bilaterally. Neuropathological examination at autopsy revealed that most of the nuclei and tracts associated with the auditory system were destroyed by lymphoid cell infiltration. It suggested that in the early stages of this disease, lymphoid cells could have involved the lower brainstem and, subsequently, in the advanced stages invaded the inferior colliculus.