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Intratympanic steroid use for hearing salvage in Vogt-Koyanagi-Harada syndrome.

Abstract

We discuss the rare case of a 68-year-old woman with Vogt-Koyanagi-Harada (VKH) syndrome and sensorineural hearing loss (SNHL) who was successfully treated with intratympanic corticosteroid injections. The patient had presented with bilaterally asymmetric (i.e., moderate and moderate to severe) SNHL, tinnitus, vertigo, and vitiligo. She received two intratympanic injections in her worse-hearing ear over the course of 1 month. Subsequent audiometry showed an immediate 5- to 10-dB improvement in her hearing across multiple frequencies, as well as a long-term improvement to near-normal thresholds. The hearing thresholds in her untreated ear remained stable. To the best of our knowledge, this is the first report of a patient with VKH syndrome who was successfully treated with intratympanic steroid application.

Introduction

Prominent auditory and cutaneous pigmentary abnormalities coexist as clinical features in several diseases. Vogt-Koyanagi-Harada (VKH) syndrome is an autoimmune condition characterized by ocular, neurologic, auditory, and cutaneous manifestations. Otologic complaints may include sensorineural hearing loss (SNHL), tinnitus, and/or vertigo.

The primary treatment for symptoms associated with VKH syndrome is systemic corticosteroids. Although no study has specifically examined the effectiveness of systemic steroids on the otologic manifestations of VKH syndrome, a benefit from their use has been suggested by the treatment of hearing loss associated with other autoimmune inner ear diseases. (1) Data on the potential utility of intratympanic steroids in autoimmune inner ear disease are limited to small case series, and no study has reported their use in VKH syndrome. (2)

We describe the case of a patient with VKH syndrome who received two intratympanic steroid injections and experienced subsequent improvement of her ipsilateral auditory symptoms. We also review the various disorders associated with concomitant auditory and cutaneous pigmentary abnormalities.

Case report

A 68-year-old woman presented with a recurrence of a fluctuating bilateral SNHL, a worsening of chronic tinnitus in her right ear, chronic fluctuating vertigo, and vitiligo. In addition to her aural symptoms of approximately 15 years' duration, she also had a history of chronic uveitis, and she was taking medications for hypertension and diabetes.

Four months earlier, the patient had been diagnosed by the senior author (S.S.C.) with VKH syndrome, and she was prescribed an oral corticosteroid for her acute right-sided hearing deterioration. With treatment, she experienced improvement in her hearing and alleviation of her uveitis-related visual symptoms. However, she also developed refractory elevations in her blood pressure and blood glucose level. These elevations could not be controlled through adjustments in her antihypertensive and diabetic medication regimens, which necessitated discontinuation of the oral steroids.

[FIGURE OMITTED]

At the most recent presentation, the patient was noted to have patches of vitiligo on her face, arms, legs, and trunk. Findings on otolaryngologic and neurotologic examinations were normal bilaterally. Audiometric testing revealed a moderate to severe SNHL in the right ear and a moderate SNHL in the left ear at the speech frequencies (figure, A). Her speech discrimination scores were 80% for the right ear and 84% for the left.

Because the patient had previously experienced adverse reactions to systemic corticosteroid administration, the decision was made to proceed with an intratympanic dexamethasone injection. A total of 0.5 ml of dexamethasone in a 10-mg/ml concentration was injected into the right middle ear for chemical labyrinthotomy via round-window membrane perfusion. The patient experienced transient dizziness but no other temporary or long-term complications.

Repeat audiometry 4 weeks following the injection showed a 10-dB improvement at 1,000 Hz. At that point, a second intratympanic injection was administered, and an audiometric examination performed 2 weeks later revealed an additional 5- to 10-dB improvement across multiple frequencies from 250 to 2,000 Hz (figure, B). Although the patient's speech discrimination scores did not substantially change (84% for the right ear and 88% for the left), she did report a subjective alleviation of the tinnitus in her right ear.

During 18 months of clinical follow-up, serial audiometry demonstrated an improvement (20 to 30 dB) to near normal in her right-sided hearing thresholds at the speech frequencies; however, the moderate SNHL persisted in her left ear. Her condition did not necessitate any additional intratympanic injections.

Discussion

Several disorders marked by prominent cutaneous pigmentary abnormalities are associated with hearing dysfunction. The pathophysiologic basis of this association is related to disease processes that affect melanocytes, which are neural-crest-derived cells found in the skin, eye, ear, and meninges. Within the skin, melanocytes are found in the basal epidermal layer; in the cochlea, they are located in the stria vascularis.

The function of melanocytes in the cochlea remains unclear. Animal models have suggested that increased pigmentation in the stria vascularis may exert a protective effect against noise-induced hearing loss. (3) Moreover, a protective role for melanin is implied by the observation that individuals with darker skin pigmentation are less susceptible to noise-induced hearing loss, presumably because of a higher density of melanin in the cochlea. (3)

The most common of hereditary disorders affecting melanocytes is Waardenburg syndrome, which is an autosomal dominant condition marked by SNHL, skin and hair hypopigmentation (piebaldism), and heterochromia iridis. These integumentary and auditory manifestations are believed to occur as the result of a failure of proper melanocyte differentiation during embryogenesis. (4)

Albinism is another hereditary disease with multiple genetic etiologies that all result in a common pathophysiologic process ofmelanocyte dysfunction and impaired or absent melanin production. Rare subtypes of ocular albinism have been associated with SNHL; deafness at birth is seen in the autosomal form, and late-onset hearing loss is seen in an X-linked variant. (4)

Vitiligo is an acquired disorder characterized by a loss of functional melanocytes, presumably via autoimmune destruction. While most patients with vitiligo do not report subjective auditory impairments, abnormal sensorineural hearing thresholds relative to control populations have been documented in some, but not all, reports. (5)

VKH syndrome is another disease that occurs as a result of an autoimmune destruction of melanocytes. It is most prevalent in nonwhite patients between the ages of 20 and 50 years. (6) Patients with VKH syndrome typically present with symptoms of aseptic meningitis initially followed by bilateral uveitis later. Dermatologic changes occur several weeks or months after the onset of the ocular symptoms, and they may include poliosis, vitiligo, and alopecia. Auditory symptoms associated with VKH syndrome occur with variable frequency; patients may experience hearing loss, tinnitus, and/or vertigo that typically coincides with the onset of ocular pathology.

The mainstay of evaluation for the auditory symptoms of VKH syndrome is audiometry, although the nature and extent of hearing loss associated with VKH syndrome has not been well described. A recent study of patients with VKH syndrome reported by Ondrey et al revealed that 5 of 24 patients had SNHL worse than what was seen in age-matched control populations reported in the previously published literature. (6) However, these 5 hearing losses exhibited no clear pattern with respect to threshold or frequency.

The most commonly used treatment for the ocular manifestations of VKH syndrome is systemic corticosteroids. Patients with SNHL associated with VKH syndrome are also typically treated with oral steroids. While no study has specifically evaluated the effectiveness of oral steroids in VKH syndrome, other patients with autoimmune inner ear disease have experienced modest but significant improvements in hearing after treatment with oral prednisone over a 4-week period. (1)

Most clinical evidence supporting the use of intratympanic steroids is derived from patients with sudden SNHL or Meniere disease. (2,7,8) Several studies have also indicated a benefit from steroids in smaller numbers of patients with autoimmune inner ear disease, although no previous study has described their use specifically for treatment of VKH-syndrome-related SNHL. (2)

Accepted indications for intratympanic steroids are as a second-line treatment when oral steroids fail to improve hearing and when systemic steroids are contraindicated. Systemic side effects secondary to plasma absorption of intratympanic steroids are theoretically possible but unlikely. No significant plasma absorption has been found in animal models, (9) and in human studies, the administration of intratympanic steroids has not increased blood glucose levels in diabetic populations. (10)

In view of the retrospective nature of the available data in the literature, no consensus has been reached on the most effective type and dose of intratympanic steroids, the number of injections, and the specific delivery technique.

In our patient, the untreated left ear served as a control for our intervention. While the hearing thresholds in her right ear improved over time following intratympanic steroid administration, thresholds remained largely unchanged in the left ear. This finding suggests that the observed improvement in our patient's right-sided hearing were not simply a reflection of the characteristic waxing and waning nature of disease severity that is associated with VKH syndrome and other autoimmune inner ear processes. Rather, it suggests that intratympanic steroid administration might have induced lasting qualitative changes in the autoimmune process affecting the patient's inner ear.

References

(1.) Niparko JK, Wang NY, Rauch SD, et al. Serial audiometry in a clinical trial of AIED treatment. Otol Neuroto12005;26(5):908-17.

(2.) Doyle KJ, Bauch C, Battista R, et al. Intratympanic steroid treatment: A review. Otol Neurotol 2004;25(6): 1034-9.

(3.) Pratt SR, Kuller L, Talbott EO, et al. Prevalence of hearing loss in Black and White elders: Results of the cardiovascular health study. J Speech Lang Hear Res 2009;52(4):973-89.

(4.) Carden SM, Boissy RE, Schoettker PJ, Good WV. Albinism: Modern molecular diagnosis. Br J Ophthalmol 1998;82(2):189-95.

(5.) Aydogan K, Turan OF, Onart S, et al. Audiological abnormalities in patients with vitiligo. Clin Exp Dermatol 2006;31 (1):110-13.

(6.) Ondrey FG, Moldestad E, Mastroianni MA, et al. Sensorineural hearing loss in Vogt-Koyanagi-Harada syndrome. Laryngoscope 2006;116(10):1873-6.

(7.) Chandrasekhar SS. Intratympanic dexamethasone for sudden sensorineural hearing loss: Clinical and laboratory evaluation. Otol Neuroto12001;22(1):18-23.

(8.) Banerjee A, Parnes LS. Intratympanic corticosteroids for sudden idiopathic sensorineural hearing loss. Otol Neurotol 2005;26(5): 878-81.

(9.) Yang J, Wu H, Zhang P, et al. The pharmacokinetic profiles of dexamethasone and methylprednisolone concentration in perilymph and plasma following systemic and local administration. Acta Otolaryngol 2008;128(5):496-504.

(10.) Kakehata S, Sasaki A, Oji K, et al. Comparison of intratympanic and intravenous dexamethasone treatment on sudden SNHL with diabetes. Otol Neurotol 2006;27(5):604-8.

Stanley Pelosi, MD; Sujana S. Chandrasekhar, MD, FACS

From the Department of Otolaryngology-Head and Neck Surgery, Vanderbilt UniversityMedical Center, Nashville, Tenn. (Dr. Pelosi); and the Department of Otolaryngology-Head and Neck Surgery, Mount Sinai School of Medicine, New York City, and New York Otology, New York City (Dr. Chandrasekhar).

Corresponding author: Stanley Pelosi, MD, Department of Otolaryngology-Head and Neck Surgery, Vanderbilt University Medical Center, 7209 Medical Center East, South Tower, 1215 21st Ave. South, Nashville, TN 37232. Email: Stanley.Pelosi@gmail.com

Previous presentation: The information in this article has been updated from its original presentation as a poster at the Eastern Section meeting of the Triological Society., Jan. 23-25, 2009; Boston.

Additional information: Informed consent was obtained from the patient per the protocol established by the Mount Sinai School of Medicine Institutional Review Board prior to the preparation of this manuscript for submission.
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Title Annotation:ORIGINAL ARTICLE
Author:Pelosi, Stanley; Chandrasekhar, Sujana S.
Publication:Ear, Nose and Throat Journal
Article Type:Report
Geographic Code:1USA
Date:Dec 1, 2011
Words:1846
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