Refractory Granulomatosis with Polyangiitis Presenting as Facial Paralysis and Bilateral Sudden Deafness.

Refractory Granulomatosis with Polyangiitis Presenting as Facial Paralysis and Bilateral Sudden Deafness.

J Audiol Otol. 2016 Apr;20(1):55-8

Authors: Kim SH, Jung AR, Kim SI, Yeo SG

Abstract
Granulomatosis with polyangiitisis [(GPA) or Wegener granulomatosis] is a multi-system disease characterized by granuloma formation and necrotizing vasculitis. GPA classically shows involvement of the respiratory tracts and the renal system. However, locoregional disease is common and may include otologic manifestations. Although otologic involvement can occur during the course of GPA, no report has described facial palsy with sudden sensorineural total deafness with vertigo as the presenting feature of GPA. This case describes a patient with multiorgan involving resistant form of GPA initially presenting with bilateral profound sudden sensorineural hearing loss and left facial paralysis with vertigo. The condition responded well to treatment with rituximab.

PMID: 27144236 [PubMed]

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Short-Term Outcomes of Acute Low-Tone Sensorineural Hearing Loss According to Treatment Modality.

Short-Term Outcomes of Acute Low-Tone Sensorineural Hearing Loss According to Treatment Modality.

J Audiol Otol. 2016 Apr;20(1):47-52

Authors: Chang J, Yum G, Im HY, Jung JY, Rah YC, Choi J

Abstract
BACKGROUND AND OBJECTIVES: We compared improvements in hearing thresholds in acute low-tone sensorineural hearing loss (ALHL) patients after two different treatments: steroid alone and steroid and diuretic combined. We analyzed how the duration between the onset of symptoms and the initiation of treatment affected hearing loss improvement and investigated the relation between presence of vertigo in ALHL patients and ALHL progression to Ménière's disease (MD).
SUBJECTS AND METHODS: We retrospectively analyzed the medical records of 47 ALHL patients aged 21 to 76 years. Patients received either orally administered steroid alone (n=12) or steroid and diuretic combined (n=35). We compared improvements in the two groups' hearing thresholds at three lower frequencies (125, 250, and 500 Hz) after participants had received one month of each respective treatment.
RESULTS: Our two treatments did not show any statistical difference in hearing loss improvement after one month. Forty percent of ALHL patients with vertigo developed MD, which was a significantly higher rate than the 12.5% of ALHL patients without vertigo who developed MD. The shorter duration between the onset of symptoms and the initiation of treatment significantly increased improvement in the sum of lower frequency hearing threshold after one month.
CONCLUSIONS: The current study suggests that steroid and diuretic administered together and steroid alone similarly improve the hearing threshold in ALHL patients after one month. We concluded that patients should initiate ALHL treatment as soon as they experience symptoms. ALHL patients should also be notified of their higher risk of developing MD.

PMID: 27144234 [PubMed]

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Labyrinthine sequestration secondary to auditory implantation: report of two cases and review of the literature.

Related Articles

Labyrinthine sequestration secondary to auditory implantation: report of two cases and review of the literature.

J Laryngol Otol. 2015 Dec;129(12):1238-42

Authors: Warner E, Eze N, Connor S, Jiang D

Abstract
BACKGROUND: Auditory implantation into the inner ear is increasingly performed for a variety of indications. Infective complications are rare, but when they occur they can have devastating consequences.
CASE REPORTS: This paper reports two cases where vestibular sequestration of the bony labyrinth developed following implantation into the middle ear.
CONCLUSION: To the authors' knowledge, these are the first reported cases where vestibular sequestration has resulted from auditory implant surgery. This paper outlines the radiological changes characteristic of this pathology. It also describes the surgical and conservative treatment options for this condition, challenging the previously accepted belief that affected patients always require aggressive surgical intervention.

PMID: 26654641 [PubMed - indexed for MEDLINE]

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