Portuguese Version

Year:  2001  Vol. 67   Ed. 2 - (16º)

Relato de Casos

Pages: 249 to 251

Sudden Deafness in Aids.

Author(s): Maria F. P. Carvalho*,
Renato Tidei**,
Fernando A. Q. Ribeiro***.

Keywords: sudden deafness, Aids, hearing loss

Abstract:
The incidence of otolaryngological diseases in Aids is very high (40 to 90%), witch hearing loss is the commonest otological manifestation that can be sensorineural or conductive, often progressive and bilateral, caused by opportunist diseases or ototoxic agents. The authors present a case of unilateral sudden deafness in a 44 year-old male patient with virus HIV, but no symptoms for four years. Besides sudden deafness, he patient didn't present any other clinical or blood tests defect, CT and MR were normal. They conclude that sudden deafness was the first and unique manifestation of Acquired Immunodeficiency Syndrome (Aids) in this patient, and suggest deep investigation in a case of sudden deafness, considering Aids as a possible cause.

INTRODUCTION

In June 1981 the first cases of acquired immunodeficiency syndrome or AIDS were reported in Los Angeles, USA, in five male homosexual patients who presented Pneumocystis carinii pneumonia and other opportunists infections11. In 1983, the agent of the pathology was defined - a retrovirus named HIV Since, then, the disease has spread all over the world and in Brazil there are approximately 30,000 notified cases, predominantly in the Southeast region6.

AIDS is characterized by states of immunosuppression that predispose the infected patient to opportunist infections, such as pneumocystosis and candidiasis, or neoplasia, such as Kaposi sarcoma. HIV has tropism for lymphocytes helper T (CD4), causing their reduction, modifying all immune response because of the reduction of interleukin 2, alteration of lymphocytes B and natural killer cells and, consequently, inactivating macrophages.

The incidence of otorhinolaryngological manifestations in adults, including the head and neck, is variable, and according to the literature it may reach 40% to 90%6. In many occasions, the first manifestations of the disease are presented in these areas.

In children, the incidence is probably even higher because of recurrent bacterial infections that are very common. According to Figueiredo7 (1999), who studied otorhinolaryngological manifestations in children with AIDS, otitis media was the most frequent ENT affection, present in 62% of the children. ENT manifestations were present in 72% of the children and the most frequent ones were: cervical lymphoadenopathy, secretory otitis media and chronic otitis media. The authors followed up 37 infected children and compared acute infections presented by them during the follow-up using immune staging and obtained the following results:

• Stage 1 (no evidence of immunosuppression) - acute otitis media

• Stage 2 (moderate immunosuppression) - acute parotiditis

• Stage 3 (severe immunosuppression) - acute sinusitis.

According to the literature, the most common otological manifestations of AIDS are hearing loss (62%), otalgia (50%), otorrhea (31%), vertigo (15%) and tinnitus (15%). Hearing losses may be conductive or neurosensorial13. The incidence of sensorineural hearing loss in AIDS patients varied from 23% to 49%12. Normally, they were progressive and bilateral and may derive from an opportunist infection or from the use of ototoxic medication taken for AIDS treatment, such as amphoterecin B, pentamidine, pirimetamine, cetoconazol, acyclovir, AZT, DDI10, and cytostatic drugs, such as cisplatin2.

Sudden deafness in patients with AIDS may be attributed to the presence of HIV virus in the auditory nerve. It has not been confirmed yet because the virus has not bee isolated in this site yet. It may also be caused by opportune; infections owing to immunodepression, such as otosyphili; toxoplasmosis, criptococcosis (Criptococcus neoformans ha already been identified in the cochlear nerve and in Corti's organ)8, encephalitis, fungal, bacterial, tuberculous or viral, (herpes) meningitis, or by central nervous system tumor, such as lymphoma or Kaposi sarcoma.

We did not find in the literature any cases of sudde deafness as the first and only AIDS manifestation.

CASE REPORT

J. G. S., 41-year-old Caucasian male, clerk, came to the Service of Otorhinolaryngology at Santa Casa de São Paulo, on April 16, 1998, referred by the infectologist from a health care unit where he was followed because of asymptomatic HIV for four years. He referred that he ha been infected by extra-marital sexual intercourse and took AZT and DDI, prescribed by the physician. He also reported sudden deafness on the left for 3 days, followed by moderat vertigo that had already improved.

He also reported syphilis, treated 10 years before denied homosexual contact and had smoked 10 cigarette a day for 25 years. The patient was married, had 3 children and gone of them had positive HIV.

Lab exams:

• Complete blood count: normal white series, normal red series, Hb and Ht, with increase of middle corpuscular volume (MCV).

•0normal rate of hemosedimentation.

• Serology for toxoplasrnosis - non-reagent.

• Serology for mononucleosis - no reagent.

• Serology for syphilis and cytomegalovirus - IgG reager and IgM non-reagent (meaning that there were serologic reactions for syphilis and cytomegalovirus).

• Anti-HB - non-reagent.

• Normal lipid analysis.

• Normal total and fraction cholesterol.

• Negative anti-nucleus and rheumatoid factors.

Head and ear CT scans were normal. Normal head MRI.

Audiometry: the patient underwent the firs audiometry when he was admitted to our service and the test showed profound sensorineural hearing loss on the left with thresholds at about 95dB.

Progression

Even under treatment with corticoids, using prednisone scheme, in decreasing doses for 16 days - 60mg for 4 days, 40mg for 4 days, 20mg for 4 days and l0mg for days - the patient did not present improvement of the auditory picture (after four months with control and audiometry) and quit treatment. During treatment, he did not present any other AIDS manifestations.

DISCUSSION

Sudden deafness is defined as a sensorineural hearing loss of quick onset that progresses rapidly in a period of hours or few days. It is normally unilateral, may be followed by vertigo (50% of the cases), tinnitus and ear fullness. Incidence according to gender is evenly distributed.

Sudden deafness may be etiologically classified in two groups8:

1) Temporal bone lesions

a) Schwanoma of the 8th cranial nerve;

b) tumor of cerebellum-pontine angle;

c) fistula of round or oval window;

d) aneurysm of anterior-inferior cerebellum artery.

2) Systemic diseases that involve temporal bone

a) viral infections that affect the cochlea (such as mumps virus);

b) blood hypercoagulability;

c) blood hyperviscosity:
polycytemia vera;
macroglobulinemia.

d) arteriosclerosis secondary to:
age;
arterial blood hypertension;
diabetes;
hyperlipidemia.

e) collagen diseases;

f) multiple sclerosis, syphilis, and others.

Even so, there are still a large number of conditions that remain undiagnosed despite comprehensive investigations and are classified as idiopathic.

Bohadana et al.1 (1998) described a case similar to ours, with sudden deafness as the main AIDS manifestation, but the patient had toxoplasmosis of the central nervous system at advanced stage and that could have been the cause of the hearing loss. In the patient in our study, sudden deafness was the first and only AIDS manifestation, because the patient did not present any other detectable affection.

In the study by Marra et al. in 199710, they investigated the effect of anti-retroviral therapy on the development of hearing loss in patients with AIDS. They studied 99 subjects submitted to anti-retroviral treatment with drugs such as zidovudine (AZT), didanosine (DDI), zalcitabine and stavudine. The results showed that the prevalence of sensorineural hearing loss in patients with AIDS submitted to anti-retrovirus therapy is significant in patients over 35 years of age. The authors believe that it is due to damage to the mitochondrial DNA. Nucleotides of anti-retroviral agents act as destroyers of mitochondrial DNA chain. We know that mutations and deletions of mitochondrial DNA are responsible for sensorineural hearing loss in syndromic and non-syndromic forms, and the mutation of mitochondrial DNA responsible for ototoxicity of aminoglycoside has been well defined'. Anti-retroviral agents may cause an alteration in energy production (ATP) in the mitochondria of the inner ear, leading to sensorineural hearing loss. The frequency of mitochondrial mutations increases with age; therefore, older subjects treated with anti-retroviral agents would be more susceptible to hearing loss. The patient in the case reported here had been submitted to treatment for 4 years with AZT and DDI. However, a factor that is not perfectly explained is that mitochondrial damage caused by these drugs normally results in bilateral and, in many situations, progressive loss5.

CONCLUSION

Since the patient had no clinical symptom, lab abnormality or imaging alteration that justified the etiology for sudden deafness, we concluded that it should have been caused by the HIV virus. Therefore, we suggested that all patients with sudden deafness with no confirmed cause shoulld be submitted to investigation of positive HIV as routine, because it may be itself a possible cause.

REFERENCES

1. BOHADANA, S. C.; LIMA, S.; MAIA, L. M. S. V; GONÇALEZ, F.; SILVEIRA, E. G. C.; JÚNIOR, O. M.; RAUSIS, M. B. G.; SILVEIRA, J. A. M. - Surdez súbita como primeira manifestação da síndrome de imunodeficiência adquirida. Rev. Bras. de Otorrinolaringologia, 64 (2): 151-56, 1998.
2. BORGES, G. C. - Análise de estudos sobre a ototoxicidade da cisplatina a sua prevenção. Dissertação de tese de mestrado, Faculdade de Ciências Médicas da Santa Casa de São Paulo, São Paulo, 1999.
3. BRAVERMAN, L; JABER, L.; LEVI, H.; ADELMAN, C.; ARONS, K.S.; FISCHEL-GHODSIAN, N.; SHOHAT, M.; ELIDAN, J. - Audiovestibular findings in patients with deafness caused by a mitochondrial susceptibility mutation and precipitated by an inherited nuclear mutation or aminoglycosides. Arch Otolaryngol Head and Neck Surg., 122: 1001-4, 1996.
4. DOMECH, J.; FUSTE, J.; TRASERRA, J. - Equilibrium and auditory disorders in patients affected by HIV 1. Rev. Neurol., 24 (136): 1623-6, 1996.
5-ENSINK, R. J. H.; CAMP, G. V; CREMERS, C. W R. J. Mitochondrial inherited hearing loss. Clin. Otolaryngol, 23: 3-8, 1998.
6. FERREIRA, N. G. M. & FERREIRA, A. G. - Manifestações otorrinolaringológicas em pacientes infectados com o vírus da Aids. Folha Médica, 105 (3): 147-156, 1992.
7. FIGUEIREDO, C. R. - Manifestações otorrinolaringológicas em crianças com síndrome da imunodeficiência adquirida. Dissertação de tese de mestrado, Universidade Federal de São Paulo - Escola Paulista de Medicina, São Paulo, 1999.
8. LEE, K. J. - Essential Otolaryngology: Head and Neck Surgery. 6th edition, Appleton and Lange, 1995.
9. LUCENTE, F. E. - Otolaryngologic aspects of acquired immunodeficiency syndrome. Medical Clinics of North America, 75 (6): 1389-98, 1991.
10. MARRA, C. M.; WECHKIN, H. A.; LONGSTRETH, W T; REES, T. S.; SYAPIN, C. L.; GATES, G. A. - Hearing loss and anti-retroviral therapy in patients infected with HIV 1. Arch. Neurnl 54- 407-111 1907
11. MORRIS, M. S. & PRASARD, S. - Otologic disease in the acquired immunodeficiency syndrome. Ear, Nose and Throat Journal, 69: 451-3, 1990.
12. RAREY, K. E. - Otologic pathophysiology in patients with human immunodeficiency virus. Am. J. Otolaryngol., 11: 366-9, 1990.
13. SALZER, T. A. - Neurotologic manifestations of HIV infection. Grand Rounds Archives, 24: 1-5; 1994.

* Postgraduate of the Discipline of otorhinolaryngology at Faculdade de Clínicas Médicas da Santa Casa de São Paulo.
** Resident of the Department of Otorhinolaryngology at Santa Casa de São Paulo.
*** Joint Professor of the Department of Otorhinolaryngology at Faculdade de Ciências Médicas da Santa Casa de São Paulo

Study presented as a poster at 34° Congresso Brasileiro de Otorrinolaringologia, in Porto Alegre/RS, in November 1998.
Study conducted at the Department of Otorhinolaryngology, Santa Casa de São Paulo.
Address for correspondence: Dra. Maria de Fátima Carvalho - Rua Hilario Furlan, 107 - Brooklin Novo - 04571-180 São Paulo/SE
Tel / Fax: (55 11) 5505-5363 / 5505-1915.
Article submitted on March 1, 2000. Article accepted on May 18, 2000.

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