Disclaimer: The information contained within the Grand Rounds Archive is intended for use by doctors and other health care professionals. These documents were prepared by resident physicians for presentation and discussion at a conference held at Baylor College of Medicine in Houston, Texas. No guarantees are made with respect to accuracy or timeliness of this material. This material should not be used as a basis for treatment decisions, and is not a substitute for professional consultation and/or peer-reviewed medical literature.

As health care providers we are faced with the prospect of caring for an increasing number of AIDS patients. The disease is no longer limited to specific populations and nearly everyone is at risk. Familiarity with the protean manifestations of AIDS is important for early diagnosis and treatment. AIDS is of particular interest to the Otolaryngologist-Head and Neck surgeon since 40-84% of patients have a symptom or physical finding in the head and neck region at initial presentation. Early diagnosis is essential because prompt initiation of treatment significantly diminishes morbidity and improves both quality and length of life in affected individuals. We will briefly review current statistics relevant to the AIDS epidemic, discuss the classification of HIV associated diseases and review in detail the neurotologic manifestations of HIV infection.

The causative agent for AIDS, HIV, is a retrovirus of the subfamily Lentovirinae. The term retrovirus is used because the virus contains the enzyme reverse trancriptase, with transcribes viral RNA to DNA- the reverse of other viral genetic transcription. HIV appears to be neurotropic and lymphotrophic and preferencially attacks T-helper cells, which are central to the function of the human cell-mediated immune system. Impairment of this system renders the host susceptible to numerous opportunistic infections from viruses, fungi, and protozoas, many of which are native to the oral cavity, pharynx and larynx. Aside from infectious consequences, HIV is also associated with malignancy, degenerative diseases and autoimmune sequelae.

OTOLOGIC-NEUROTOLOGIC MANIFESTATIONS
Otologic complaints are common in AIDS patients and include: hearing loss (62%), otalgia (50%), otorrhea (31%), vertigo (15%) and tinnitus (15%). It is not known if the neurotologic effects seen in AIDS is the effect of HIV alone or a combination of the effects of HIV infection coupled with opportunistic infections and/or possible toxic effects of certain therapeutic agents. The brain may be the site of tumors such as lymphoma or metastatic Kaposi's sarcoma. The HIV itself may cause aseptic meningitis, subacute encephalitis and isolated cranial nerve neuropathy. The following case reports associate HIV infection with otologic diagnoses:

RAMSAY HUNT SYNDROME
Mishell and Applebaum 1990, reported a case of a 29 y/o homosexual male who was HIV+ with Ramsay Hunt syndrome associated with facial nerve palsy. The patient was treated with intravenous nafcillin and acyclovir.

HERPES ZOSTER MYRINGITIS
Kohan et al 1988 reported two cases of Herpes zoster myringitis which were successfully treated with acyclovir.

KAPOSI'S SARCOMA
KS is a slowly progressive, malignant mesenchymal tumor characterized clinically by red-purple plaques and nodules that may be seen externally or internally. Proliferation of atypical spindle cells within vascular channels is seen on histopathology. KS has been reported to involve the mastoid (Linstrom 1993) and external ear (Morris 1990).

GRADENIGO'S SYNDROME
Linstrom et al 1993 reported a 28 year old male IVDA with otalgia, purulent otorrhea, and sixth and seventh cranial nerve palsy secondary to petrous apicitis due to Aspergillus infection

PROGRESSIVE MULTIFOCAL LEUKENCEPHALOPATHY
Langford and Kuntz 1988 presented two adult patients with AIDS who had multiple cranial neuropathies resulting from progressive multifocal leukencephalopathy. Clinical symptoms included facial paralysis, trigeminal hypaesthesia, hemianopsia, and deafness. Cranial MRI in both patients showed progressive PML, an unusual demyelinating disease seen in AIDS patients and thought to result from a direct effect of HIV in the cerebral white matter.

PNEUMOCYSTIS CARINII OTITIS / MASTOIDITIS
Pseudomonas carinii is the most common opportunistic pathogen infecting persons with HIV infection, affecting nearly 85% patients at some point of the illness. Otic Pneumocystis typically presents as a unilateral polypoid mass associated with otalgia, hearing loss and occasionally otorrhea. Pneumocystis carinii otitis media and mastoiditis have been. Pneumocystis carinii recovered from aural polyps is occasionally the initial manifestation of HIV infection in patients presenting to otologists for evaluation of otorrhea, otalgia and hearing loss. Pneumocystis carinii of the external canal has been effectively treated with local debridement and a 3- week course of Bactrim.

OPPORTUNISTIC INFECTIONS
Toxoplasmosis is the most common opportunistic infection to effect the CNS (Real, 1988). Kohan reported two such patients which presented with bilateral SNHL. Cryptococcal meningitis is known to be associated with SNHL in up to 25% cases (Real, 1987). Tuberculous meningitis is not infrequent in AIDS patients and is known to be associated with hearing loss.

OTOSYPHILIS
Otosyphilis must always be considered and investigated in a patient with fluctuating, asymmetric or sudden hearing loss. The diagnosis and treatment of syphilitic cochleovestibular dysfunction remains controversial, however the presumptive diagnosis is made in patients with cochleovestibular dysfunction, positive serology for syphilis and no other known etiology for the inner ear disturbance.. Smith and Canalis, 1989 found otosyphilis to develop at an accelerated rate from primary syphilis in AIDS patients. All cases arose in patients previously treated for primary syphilis (latent syphilis). They propose that the HIV somehow hastens the development of otosyphilis effectively activating the disease.. Furthermore they propose that all patients with otosyphilis should have an HIV test.

The index of suspicion should be high in the homosexual population because of the high incidence of syphilis in this group. 35-55% patients with AIDS have a past history of other sexually transmitted diseases including syphilis. Otosyphilis is well recognized as a cause of otologic disease in patients with AIDS (Morris and Prasad, 1990).

Gleich et al identified three factors associated with hearing improvement in patients with otosyphilis treated with intravenous penicillin and corticosteroids. In there study 31% patients with otosyphilis experienced hearing improvement, tinnitus decreased in 85% and vertigo improved in 86% . Specific factors associated with hearing improvement include: hearing loss present less than 5 years, fluctuating hearing, and age less than 60 years. Improvement was unrelated to the severity of the loss or previous treatment.

CENTRAL NERVOUS SYSTEM
HIV is frequently accompanied by neurological complications which are observed in 40% to 75% of patients during the course of the disease. De la Monte discovered CNS involvement in more than 90% of HIV infected patients at autopsy. Neurological findings are detectable in approximately one third of AIDS patients. At autopsy however, up to of 75% subjects reveal neuropathological changes related to HIV infection. The most common neurologic syndrome seen in AIDS patients is subacute encephalitis. Early stages are characterized by subtle cognitive changes that may progress to dementia in several weeks or months. The HIV appears to be directly involved in both clinical and pathological changes in the CNS. Neurotologic workup may reveal central vestibular and auditory dysfunction. ENG findings show ataxic pursuit and optikinetic nystagmus with total loss of caloric excitability. ABR indicate absolute and interpeak latencies and synthetic sentence identification test yield reduced scores. At autopsy the AIDS virus was found in mononuclear and multinuclear giant cells in the cortical and subcortical gray matter, cerebral and cerebellar white matter and through out the brain stem.

Hausler compared the incidence of peripheral and central auditory and vestibular disorders occurring in different stages of HIV infection. The results of audiological, vestibular and electrophysiologic tests performed on symptomatic and asymptomatic HIV-positive homosexual males with age matched seronegative homosexual males were compared. 57% of symptomatic and 45% off asymptomatic HIV positive patients had substantial abnormalities in comparison to minor abnormalities detected in 12% seronegative patients. The results suggested a high incidence of neurotologic disorders in HIV positive patients and that subclinical involvement of the auditory and vestibular system is common.

The frequency of electrophysiologic abnormalities in asymptomatic patients indicates subclinical involvement of the Central nervous system.

SENSORINEURAL HEARING LOSS
The incidence of SNHL in patients with HIV ranges from 23 - 49% . Kohan reported seven patients with persistent SNHL. In three instances the hearing loss could be traced to the use of well-known ototoxic drugs used in the treatment of AIDS. The remaining four patients were divided among CNS toxoplasmosis and cryptococcal meningitis, CNS toxoplasmosis alone, tuberculous meningitis and leutic otitis. For example, acyclovir may cause vertigo, trimethoprim-sulfamethoxazole may cause vertigo and tinnitus, and azidothymidine may lead to vertigo and hearing loss.

The characteristics of the SNHL in patients with HIV are not uniformly defined. The degree of hearing loss, range of loss and even incidence appear variable. Hearing loss in the high frequencies is more commonly reported than in the low frequencies. Delayed latencies of brainstem auditory-evoked responses have been observed in patients with HIV, suggesting neuropathies of the central auditory and vestibular region.

Boccellari found an association between immune suppression in HIV patients and neurophysiologic measures. Asymptomatic subjects without evidence of immune suppression do not appear to be at greater risk for neurophysiological impairment than HIV-negative subjects. The HIV-positive individuals with evidence of immunosuppression, however, appear to have an increased likelihood of central conduction time slowing. In contrast, studies by Pagano suggest that subclinical involvement of the upper brain stem occurs in HIV infection and that brainstem auditory evoked potential abnormalities could be the direct result of the HIV effect on central nervous system structures.

Case Presentation

A 35-year-old man with human immunodeficiency virus infection diagnosed seven years ago was hospitalized for disseminated histoplasmosis which served as his AIDS-defining illness. During that admission he was also found to have Mycobacterium avium-intracellulare septicemia and bilateral external otitis secondary to Pseudomonas aeruginosa. CT scan of the temporal bones and gallium scan failed to reveal bony involvement. The otitis externa was treated with local debridement, culture specific intravenous antibiotics and otic drops. The patient experienced near total resolution of his symptoms with decreased otalgia, erythema and edema. Post treatment cultures were negative. Two months later he presented to the Otolaryngology service for evaluation and treatment of recurrent otitis externa which had progressed despite oral ciprofloxacin and Cortisporin otic drops. Examination revealed severe external otitis with erythema, edema and ulceration limited to the concha cavum and a right paranasal ulcer. The possibility of drug reaction was entertained and initial improvement was seen with withdrawal of the otic drops, however symptoms worsened thereafter prompting tissue biopsy of the ear and paranasal region for histology and culture. Histologic evaluation revealed multiple rrport inclusion bodies consistent with Herpes simplex infection. Audiometric evaluation was normal. The patient's condition improved dramatically on intravenous acyclovir.

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