INTRODUCTION

In children, there is lower period of latency between the acquisition of acquired immunodeficiency virus (HIV) infection and the onset of symptoms, but shorter survival after the onset of the disease 1.

HIV infects children and adolescents through three main contamination routes: maternal transmission (the most common one), blood transfusion and high-risk sexual behavior 2.

Vertical transmission from the mother to the child can be classified into three categories: intrauterine, perinatal and post-natal 2.

Transplacental transmission can occur early during pregnancy. Intrapartum transmission is the result of the contact of the child's blood with secretion from the infected genital tract of the mother. There can also be transmission of HIV through breastfeeding. In the United States, breastfeeding is not recommended for HIV positive mothers 2, 3.

Otological diseases are particularly common in children with HIV. About 40% of the patients present this type of manifestation 4-9.

Conductive hearing loss is in general resultant from secretory otitis media (SOM) secondary to the obstruction of the auditory tube, by proliferation of adenoid lymphoid tissue, or even by Kaposi's sarcoma in the rhinopharynx. SOM is normally refractory to clinical treatment and requires the conduction of tympanotomy to place ventilation tubes in children younger than 7 years, which is quite frequent. Recurrent serous effusions and subsequent hearing loss can lead to speech and language deficit 1, 5, 7, 9-11.

External otitis are uncommon in AIDS patients, representing an incidental otological disease, caused by the same agents that cause it in immunocompetent subjects rather than being manifestation of the acquired immunodeficiency syndrome (AIDS) 5.

In AIDS patients, acute otitis media (AOM) is normally caused by Streptococcus Pneumoniae or Haemophilus influenzae. The incidence of AOM is no higher in AIDS patients than in healthy people, and the treatment with oral antibiotics normally proves to be effective 1, 5, 9, 11.

In the pediatric AIDS population, especially in children younger than 7 years, there is high incidence of recurrent acute otitis media and chronic otitis media with ossicle chain erosion, possibly owing to auditory tube immaturity. AOM should be treated aggressively to prevent complications that would be potentially fatal (sepsis, for example) 1, 2, 5.

There are no studies showing that chronic otitis media, with or without cholesteatoma, is more prevalent in HIV positive patients. In general, etiopathogenesis, evolution and treatment are the same as in the non-HIV population 5, 11.

Many cases of otomastoiditis caused by Pneumocystis carinii have been reported in AIDS patients. The probable route of infection is retrograde, through the auditory tube, after the asymptomatic colonization of rhinopharynx. The clinical presentation includes otalgia, mixed deafness and aural polyps. Biopsy of the polyps reveals pathogen typical cysts. Treatment can be made using sufametoxazole-trimethoprim for 10 to 21 days4,5,7,9,11.

There are potential causes of sensorineural hearing loss in AIDS patients and the more severe the disease manifestations, the more severe the loss.

The immunodeficient status of the patients predisposes to meningeal infections that can lead to sensorineural hearing loss, including meningitis caused by Cryptococcus neoformans, meningeal tuberculosis and viral, fungal and bacterial meningitis. As extension of meningitis, these organisms can cause labyrinthitis and lead to hearing loss. Neurotoxoplasmosis, cytomegalovirosis and herpes infections affect the spiral ganglion and cochlear branch of 8th nerve and can lead to deafness.

Intracranial events such as encephalitis, progressive multifocal leukoencephalopathy, hemorrhage and cerebral lymphoma can also lead to hearing deficit 1, 5, 7, 9-13.

HIV, in addition to its lymphotropic behavior, has neurotrophism as well. Therefore, it is believed that the virus affects the 8th nerve and can lead to sensorineural hearing loss.

However, the effects of HIV on the vestibular-cochlear nerve are not completely understood yet, since it has never been isolated in nerve or spiral ganglion cells 5, 8, 10.

Otosyphilis is a known cause of sensorineural hearing loss and this disease has increase prevalence in HIV patients. Otological affection by Treponema pallidum takes place on tertiary Lues' disease. The initial symptoms are both uni or bilateral and quickly progressive hearing loss, which can sometimes be sudden. Tinnitus, aural pressure and labyrinthic symptoms are also present. The audiometric curve normally shows decrease in low frequencies, suggestive of endolymphatic hydrops. The diagnosis is made through serologic tests such as VDRL and FTA-ABS and treatment with benzatin penicillin 2,400,000 UI IM, once a week, for 2 weeks, with a week interval, associated with corticoids5, 11, 13.

AIDS patients use many drugs to treat opportunistic infections, which in many occasions cause ototoxicity. The drugs in question are, among others, anphotericin B, sulfas, pentamidine, pirimetamine, ketokonazole, acyclovir and aminoglycosides 5.

After six months of therapy, drugs that are part of the anti-retroviral cocktail, such as AZT, zidovudine, didanosine and stavudine, in general lead to sensorineural deafness by ototoxicity 14.

A possible mechanism that causes hearing loss in patients taking anti-retroviral treatment is mitochondrial DNA defect in hair cells. The anti-retroviral agents can impair the production of energy by mitochondria in the inner ear. DNA mitochondrial abnormalities accumulate with aging and, therefore, older subjects are more susceptible to this type of lesions 14, 15.

The follow-up of AIDS patients concerning hearing loss should follow the routine proposed by Sancho et al.8:

1. We start by conducting detailed ENT examination, especially through well conducted otoscopy. Next, we should order pure tone audiometry, speech discrimination and acoustic immittance tests, provided there is no tympanic membrane perforation;

2. In cases of conductive hearing loss, nasal endoscopy and/or paranasal sinuses x-rays should be performed to assess nasal fossa and especially rhinopharynx. Temporal bone CT scan and MRI are requested for selected cases in order to better visualize the extension of the disease.

3. In cases of perceptual hearing loss, evoked otoacoustic emissions are investigated, as well as brainstem auditory response to assess whether the lesion is cochlear or retrocochlear.

CT and MRI help to determine the diagnosis in selected cases. We should investigate through serology tests the presence of previous history of some specific etiological agent causing hearing loss.

In ABR of HIV positive patients with sensorineural hearing loss, we normally find increased latencies of waves I and V and III and V.

Children with conductive hearing loss, after being properly diagnosed, should be treated accordingly (antibiotics, ventilation tubes, adenoidectomy, etc) and those with perceptual hearing loss, many times require the use of hearing aids and specialized speech and hearing therapy.

CASE REPORT

DAS, 10 years of age, female Caucasian, complaining of deafness for 8 months. Her accompanying person referred that from infant to the age of 3 years she had had repetitive episodes of otalgias, followed by right ear otorrhea. Eight months before she presented progressive hearing loss, worse on the right. She did not report pain, otorrhea, tinnitus and dizziness in the period. No nasal or oropharyngeal complaints and no language deficits. She had had positive HIV for two years and had started taking anti-retroviral cocktail with D4T, 3TC and took sulfametoxazole-trimethoprim for prophylaxis of upper airway infections.

Her relevant personal history included repetitive episodes of upper airway infections, three episodes of meningitis, many episodes of bronchopneumonia, herpes zoster on the trunk and onicomycosis in both feet. Parents had abandoned her at 20 days of life. The relatives did not report hearing loss in the family. She had been wearing a hearing aid for 1 month on the left and seemed to have adapted quite well.

The physical examination presented central wide perforation of the tympanic membrane, with erosion of the distal portion of the malleus region on the right and no affection on the left. Rhinoscopy showed hypertrophic and pale inferior turbinates ++/4; septal deviation grade I in area IV to the left, normal oroscopy. All the other organs did not show any other abnormalities.

Nasofibroscopy showed increase in volume of lymphoid tissue in rhinopharynx, occupying 80% of its lumen.

Pure tone audiometry presented moderate sensorineural hearing loss on the left and mixed severe to profound hearing loss on the right (Figure 1).

Serology tests for toxoplasmosis, herpes, rubella, VDRL and FTA-ABS were all negative. Serology for cytomegalovirus IgG was positively reagent.

Temporal bone CT scan showed hypoplastic mastoid on the right, without signs of labyrinth ossification and no other relevant findings.
ABR did not produce results suggestive of retrocochlear disease.

DISCUSSION

Otological involvement is common in children with AIDS. In the case described here we addressed the history of a 10 year-old child with severe sensorineural hearing loss, rapidly progressive, bilateral and symmetrical, followed by conductive component on the right ear.

The conductive component related to the fact that she had presented repetitive suppurated acute otitis media during her whole childhood years, always on the right ear. Since at nasofibroscopy she presented hypertrophic adenoids, signs of allergic rhinopathy and sinusopathy, it is suggested that she had had a deficit of ventilation in the middle ears, and consequently, maintained secretory otitis media in between the acute episodes of otitis up to the moment in which there was definite perforation of the right tympanic membrane and conductive hearing loss.

Sensorineural hearing loss has many possible factors to explain it. Among them, previous infection or cytomegalovirus (which can take to spiral ganglion lesion), right ear neural impairment by HIV (less probably owing to the fact that ABR did not suggest retrocochlear lesion), damage to hair cells because of the use of ototoxic drugs and labyrinthic lesion secondary to the action of toxins of bacteria involved in chronic otitis media and meningitis presented by the child.

CONCLUSION

IT is important that children with HIV be referred to complete ENT examination since it is very common to find involvement of the auditory system, as well as the nose, paranasal sinuses, salivary glands, pharynx and larynx in AIDS cases.

From an otological perspective, children should follow the protocol as the one suggested by Sancho et al.8, to try to determine the cause of the problem and treat it.

Diagnosis, especially in cases of sensorineural hearing loss, is not always easy, but early detection of hearing loss is important to better control progression of the disease and indication of therapy and speech and therapy rehabilitation.

REFERENCES

1. Chen AY, Ohms LA, Stewart MG, Kline MW. Otolaryngologic Disease Progression in Children with Human Immunodeficiency Virus Infection. Arch Otolaryngol Head Neck Surg 1996; 122:1360-3.
2. Chow JH, Stern JC, Kaul A, Pincus RL, Grosmich DS. Head and Neck Manifestations of the Acquired Immunodeficiency in Children. Ear, Nose and Throat Journal 1990; 69:416-23.
3. Sculerati N, Borkowsky W. Pediatric human immunodeficiency virus infection: an otolaryngologist's perspective. J Otolaryngol 1990; 19(3):182-8.
4. Chandrasekhar SS, Connely PE, Brahmbhatt SS, Shah CS, Kloser PC, Baredes S. Otologic and Audiologic Evaluation of Human Immunodeficiency Virus-Infected Patients. Am J Otol 2000; 21(1):1-9.
5. Ferreira NGM, Ferreira AG. Manifestações otorrinolaringológicas em pacientes infectados com o vírus da AIDS. F Med (BR) 1992; 105(3):147-56.
6. Munhoz MSL, Uip DE, Munhoz ALL, Munhoz MLGS. Manifestações otorrinolaringológicas em paciente com síndrome da imunodeficiência adquirida (Sida). RBM - Otorrinolaringologia 1994; 1:54-7.
7. Rothstein SG, Kohan D, Cohen NL. Otologic Disease In Patients With Acquired Immunodeficiency Syndrome. Ann Otol Rhinol Laryngol 1988; 97:636-9.
8. Sancho EM, Dominguez AU, Urpegui JM, Jimenez SB, Valles H. Hipoacusia perceptiva y SIDA. Rev Neurol 1997; 25(142):887-90.
9. Truitt TO, Tami TA. Otolaryngologic Manifestations Of Human Immunodeficiency Virus Infection. Med Clin North Am 1999; 83(1):303-8.
10. Lucente FE. Otologic Aspects of Acquired Immunodeficiency Syndrome. Med Clin North Am 1991; 75:1389-97.
11. Morris MS, Prasad S. Otologic Disease in the Acquired Immunodeficiency Syndrome. Ear Nose and Throat Journal 1990; 69:451-3.
12. Rarey KE. Otologic Pathophysiology in Patients With Human Immunodeficiency Virus. Am J Otolaryngol 1990; 11:366-9.
13. Youngs R. Human immunodeficiency virus in otolaryngology. J Laryngol Otol 1997; 111:209-11.
14. Marra CM, Wedkin HA, Lonstreth Jr WT, Ries TS, Syapiun CL, Gates GA. Hearing Loss and Retroviral Therapy in Patients Infected with HIV-1. Arch Neurol 1997; 54:407-9.
15. Soucek S, Michaels L. The Ear in the Acquired Immunodeficiency Syndrome: Clinical and Audiologic Investigation. Am J Otol 1996; 17:35-9.

1 Resident physician in Otorhinolaryngology, Medical School, Fundação do ABC.
2 Resident physician in Otorhinolaryngology, Medical School, Fundação do ABC.
3 Faculty Professor, Discipline of Otorhinolaryngology, Medical School, Fundação do ABC.
Affiliation: Discipline of Otorhinolaryngology, Medical School, Fundação do ABC - Av. Príncipe de Gales 821 Santo André SP Brazil 09060-050 / Tel (55 11) 4438-3347.
Address correspondence to: Rubens E. C. Rodrigues - R. Estrada Velha da Penha 88 ap. 72 bloco 2 03090-020 Tatuapé - Sao Paulo SP.