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Hearing Loss in a Patient With Congenital HIV &… : Oncology Times – LWW Journals

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Marquess, Ali BA; Sprouse, Brittney AUD
More than 36,000 people were diagnosed in 2019 with HIV.5 It can take months or years before HIV infection might develop into AIDS.5 Unless a person gets tested, that individual may never know they are infected with HIV until they get sick/start showing symptoms.6
The current treatment for HIV is in the form of antiretroviral therapies/treatments (ART).5 These can lower viral load over time and decrease the chance of passing the virus to others, and some drug regimens are deemed safe during pregnancy.5 Cytomegalovirus (CMV) is more common in mothers who have untreated HIV.7 The most common treatment for HIV+ patients/mothers is highly active antiretroviral therapy (HAART).7 This is a highly effective drug cocktail typically comprised of three or more drugs.5 The effectiveness of HAART is variable and mostly depends on the patient’s age and initial CD4 load.14
For patients who are pregnant, HAART is critical in the prevention of mother-to-child transmission.5 CMV is also more common in mothers who have untreated HIV,7 and infants who are CMV-positive at birth (congenital CMV or cCMV) were more likely to be born to mothers who were diagnosed with HIV during the pregnancy or at delivery.4
While it is known that sensorineural hearing loss is a symptom of CMV,6 “the relationship of the effects on the auditory system from the HIV medications taken by the mother during pregnancy are less understood at this time. Currently, the mother-to-child-transmission rate is very low, given that the mother begins treatment for her HIV as soon as possible, resulting in more children born who are HIV-exposed but uninfected.”11
However, with even low viral loads at birth, mother and baby are often given RVTs during pregnancy.7 Both a cesarean section and bottle feeding are recommended to prevent future spread of the virus.7 If guidelines are followed, 99 percent of HIV-infected women will not pass HIV on to their children.7
Children infected with HIV are about 200-300 percent more likely to have hearing loss by age 16 compared with national averages for noninfected children of the same age.3 However, these percentages dropped significantly (20%) for children whose mothers are HIV+ during pregnancy, but did not pass along the virus to the infant.3 We also know given the immunosuppressive nature of HIV that HIV+ patients are more likely to develop otitis media (OM), or middle ear infections.8 In an HIV+ child, OM must be treated aggressively as further complications could arise in this population.8
The question for this case report becomes a chicken-or-the-egg scenario. Does this child with a medulloblastoma, who presents with congenital HIV and CMV, lose her hearing solely due to ototoxic effects of her cancer medications or was she predisposed to losing her hearing due to other concomitant diagnoses?
A 6-year-old female presents to the emergency room with headache, neck stiffness, neck pain, and ataxia after being put on antibiotics a week prior for a non-meningitis bacterial infection. The patient’s medical history is complex as she was born to an HIV+ mother at 29 weeks, whose self-reported viral load was low enough for a vaginal delivery. The patient was diagnosed at birth with ambiguous genitalia and congenital CMV, but negative HIV.
The patient reportedly contracted HIV through breastfeeding months later and now receives HAART. An MRI (5/25/18) confirms a large hemorrhagic venous mass with effacement. A biopsy of mass was performed days later (6/7/2018) and confirmed medulloblastoma (6/10/18), which was removed immediately (6/11/2018). The patient was then placed on six rounds of cancer treatment, including radiation and chemotherapy. Chemotherapy medication included vincristine, cisplatin, carboplatin, and cyclophosphamide.
Due to the ototoxic effects of these drugs, the patient was referred to audiology for ototoxic monitoring. The patient was seen as an infant in audiology due to cCMV, which documented present distortion product otoacoustic emissions (DPOAE) bilaterally (2,000-8,000 Hz) with the recommendation to continue monitoring for changes. With medulloblastoma diagnosis, the patient was seen at the end of each chemotherapy round (monthly).
The cause and prognosis of this child’s hearing loss is hard to determine given her complex medical history—specifically cCMV, congenital HIV exposure, and ototoxic cancer treatment. We know all of the aforementioned diagnoses have associated hearing loss. The unknown answer is whether the congenital and perinatal exposures caused this patient to become more prone or at risk for developing hearing loss from her chemotherapies, and if it will continue to progress or remain stable now that she has finished her treatment and is in remission.
Due to her mother’s positive HIV diagnosis and active RVT therapy, studies show the patient is more likely to have delayed development in language as the impact of RVT exposure in utero is still widely unknown. However, some studies have shown some ARV medications may have potential ototoxic effects and cause hearing loss, tinnitus, and dizziness.2 Language patterns of RVT-exposed infants are also being studied, and some studies are showing lower receptive language scores among this population.2 A significant association between exposure to atazanavir and language/developmental delays has also been found.10 These odds were increased if RVT exposure occurred during first trimester of pregnancy.10
“These results highlight the importance for audiologists to be knowledgeable of in utero ARV exposures in HEU (HIV-exposed uninfected) children because of the possibility of higher referrals in these children.”10 Based on this perinatal exposure to these RVTs should we, in our field, consider adding these children to our watchlist for speech, language, and hearing development—or is one hearing screening at birth enough until school age.
We also know this family has been lost to follow-up care at our center in all medical modalities dating back to her first audiological recommendations due to cCMV/HIV diagnosis. Once the patient was established with oncology several years later, serial audiograms were able to be obtained. It is here that this family may associate our office and sound booth with those troubling times of chemotherapy treatment. It is important for us as providers to understand the reasons behind patients lost to follow up and approach these situations with trauma-informed care.13
In terms of this patient and her family, the cancer diagnosis and chronic visits to the emergency department at our location may have been triggering or re-traumatizing to this family.13 The manner in which we view and respond to individual trauma (that of both patients and staff) sets the stage for the impact of trauma, as well as the facilitation of the healing/recovery process for our patients.13
ALI MARQUESS, BA, is the current 4th-Year Pediatric Extern at Comer Children’s Hospital (UChicago Medicine). She is in the Doctorate of Audiology program at the University of Illinois. BRITTNEY SPROUSE, AUD, is Director of Audiology and a pediatric audiologist at UChicago Medicine.
You can explore all the references for this article online at https://bit.ly/3MXGdxP.
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