RESUMO
Objective: Scleroderma is a complex chronic progressive immune-mediated disease that causes fibrosis of the skin and internal organs, and vasculopathy.Ear involvement has been poorly studied in patients with scleroderma. Vasculitic and autoimmune mechanisms are considered as possible etiologies on hearing impairment, however, this etiology still unclear.Herein, we reviewed three cases of scleroderma from a temporal bone repository. Methods: The national temporal bone database was reviewed for cases with scleroderma. Clinical case review and correlative otopathologic analysis. Middle and inner ear otopathologic analysis was performed following hematoxylin and eosin staining under light microscopy. Findings were compared to three age-matched controls. Results: Two patients (three cases) with a history of serologically confirmed scleroderma were identified. Both individuals reported tinnitus and demonstrated bilateral moderate to severe down-sloping sensorineural hearing loss on audiometry. Histologically, the incudomallear joint space was diminished and ossicles appeared demineralized. A loss of hyaline cartilage, and obliteration of the incudomallear and incudostapedial joint synovial spaces was observed. Decreased caliber and intimal hyperplasia of arteries adjacent to ossicles was also identified. Mild diffuse atrophy of stria vascularis in the middle and apical turns of cochlea were found. Hair cell populations were normal. Total spiral ganglion neurons were lower in cases of scleroderma (range 29%-51%) compared to age-matched controls. Conclusion: Fibrosis, inflammation, and vascular changes were observed in the middle and inner ear in patients with scleroderma. Findings have implications for understanding hearing and vestibular dysfunction in this patient population. Level of evidence: Retrospective study.
RESUMO
OBJECTIVES: Universal mask wearing due to COVID-19 has introduced barriers to clear communication. In hearing impaired individuals this can impact informed surgical consent. For cochlear implant candidates, who do not rely on sign language, real-time transcription with a stenographer (CART) is the gold-standard in assistive technologies. If CART is not available, speech to text (STT) applications have been advertised as solutions, but their transcription accuracy with or without an N95 mask is not well-established. Herein, we sought to investigate the transcription accuracy of three STT solutions for iPhone and compare their performance to the CART service at our institution. METHODS: Three native English speakers and three non-native English speakers read two passages (a cochlear implant consent and the non-medical 'Rainbow passage') with and without an N95 mask. Error rates from the comparison of the transcript (from either the STT app or CART) with the original passage were calculated. RESULTS: The CART service had the lowest error rate of all testing conditions (4.79-7.14%). Ava 24/7 (15.0 ± 9.49%) and the iPhone dictation (15.6 ± 6.65%) had significantly lower average error rates than the Live Transcribe (37.7 ± 20.3%) (P < 0.0001) application. Neither the presence of an N95 nor the type of passage had a statistically significant impact on the error rate. CONCLUSION: CART should be used to augment communication with patients who are hard of hearing. If CART is not available, a STT application such as Ava 24/7 or the native iPhone dictation application may be considered, even in the context of medical terminology.