Representatives of the American Speech-Language Association (ASHA) and the International Association of Physicians in AIDS Care (IAPAC) met October 16, 1998, in Chicago, to determine next steps in raising awareness among community physicians and HIV specialists about the special challenges to HIV patients with communication disorders. As a result of the meeting, which included a discussion of communication disorders among adults and children with HIV, ASHA and IAPAC agreed to form a committee to advance the necessary research in HIV-associated communication disorders that would provide a matrix to bring more audiologists, otologists, otolaryngologists, and speech-language pathologists into the healthcare team providing the comprehensive HIV/AIDS management.
Gregory L. Weimann, director of ASHA's Special Projects Branch, opened the meeting by citing the communication disorders most commonly reported in persons with HIV disease by ASHA's members (Table 1). He then categorized these disorders by their causative factors: disorders (1) originating prior to HIV infection, (2) caused and/or aggravated by HIV-associated opportunistic infections, (3) caused by the neurological complications of HIV disease, and (4) caused/and or aggravated by HIV drugs and/or drug interactions.
|Table 1. Common HIV-associated communication disorders reported by ASHA members|
||Language of confusion*|
||Pragmatic language disorder|
|* Pediatric disorders|
A.U. Bankaitis, PhD, CCC-A, director of audiology at the Health Services Division of St. Louis University Medical Center, commented on the difficulty in having community-physicians and HIV specialists both recognize auditory disorders in patients and refer patients with these symptoms to the appropriate specialists for evaluation and treatment. Bankaitis speculated that this difficulty may have contributed to the soft statistics on the incidence of these disorders in HIV disease which could range anywhere from 20 to 49 percent, "depending who you read."
Bankaitis said that she was concerned that HIV-associated auditory defects may have been seriously under-reported. There are anecdotal reports suggesting that hearing loss and dizziness which are often the initial symptoms of underlying auditory system disease may not have been reported by patients prior to highly active antiretroviral therapy (HAART) because many patients focused on the life-threatening complications of HIV disease rather than on quality of life issues. People who have benefitted from HAART may now become more conscious of symptoms of these disorders.
Gordon Nary, IAPAC's executive director, responded that to alert more physicians of these problems, investigators should submit their research to leading international AIDS conferences. Nary offered IAPAC's assistance in "priming the conference pump" by including a special abstract category for HIV-associated communication disorders at IAPAC's first annual members conference, which is tentatively scheduled to take place in Chicago in April 2000. He also offered to establish a special peer-review committee for original communication disorders research and papers for publication in the Journal of the International Association of Physicians in AIDS Care beginning in the second half of 1999.
Bankaitis described two kinds of hearing loss. Sensory neural loss is a dysfunction of the cochlea and/or auditory nerve which often results in patients' inability to hear or distinguish softer sounds that unaffected individuals are able to hear. Thus, the speech that they hear is often distorted. Some of the more common causes of cochlea and auditory nerve disease are cytomegalovirus (CMV), cryptococcosis, bacterial meningitis, toxoplasmosis, syphilis, and herpes zoster.
In conductive hearing loss, otologic infection affects the pinna, the outer ear, external auditory canal, tympanic membrane, and the ossicles behind the tympanic membrane. The most common cause of conductive hearing loss in the HIV population is otitis media. There is also a higher incidence of otitis media in HIV- infected individuals than in those who are not HIV-infected. (Figures 1 and 2). In addition to the routine causes of otitis media in the general population, Bankaitis explained that there has been a higher incidence of nasopharyngeal polyps and subcutaneous cysts in patients with HIV disease compared to the non-infected population. These nasopharyngeal masses can occlude the Eustachian tube and block ventilation which can contribute to the development of chronic otitis media. Surgical removal of the polyps and cysts is usually indicated. Mastoiditis can also cause conductive hearing loss and can be treated with antibiotics and surgery.
Otitis externa, an infection of the cartilaginous portion of the external auditory canal, is another common cause of conductive hearing loss. It is often found in patients with Kaposi's sarcoma lesions of the outer ear, and in patients with herpes zoster. Symptomatic treatment includes antibiotics (oral and ear drops) and steroid eardrops.
Bankaitis expressed concern over the response by some physicians that the potential risk of hearing loss due to ototoxicity is so minimal that there is no need to screen for symptoms of hearing loss. She pointed out that: (1) there have not been appropriately designed trials to determine the risk of hearing loss and other communication disorders in people with HIV disease; (2) the questionable pre-HAART data may not be applicable to those people with HIV disease whose survival has been significantly extended and in whom new manifestations of the disease and side effects of more complex therapies are increasing; and (3) audiologists rarely serve as clinical trial consultants, thus the collection of data on patient responses to symptoms of hearing loss could be biased by not asking the questions according to audiologist established standards for symptom screening.
Nary stated that a commonly proven adage in clinical medicine is "once you start looking for something, you will most likely find it." If physicians are not screening for communication disorders, then they most likely will not identify and report them, he explained.
One of the tools that audiologists use to assess audiologic disorders is the auditory brainstem response (ABR). The ABR is the bioelectric response generated by the auditory nerve in various brainstem nuclei up to the rostral pons in the middle of the brainstem. The ABR is a measure that reflects peripheral and central auditory nerve conduction. Audiologists use the technology to rule out retrocochlear pathology.
Laurel Christensen, PhD, CCC-A, an audiologist at Etymotic Research, noted that European physicians appeared to be more progressive than US physicians in using ABR in HIV patients. Christensen noted that some European physicians were using ABR to monitor the effectiveness of antiretroviral drugs. It was her impression from presenting at European meetings that ABRs were almost a standard of care in Europe whereas in the US most of the tests appear to be conducted as a component of clinical research studies. Christensen speculated that one possibility for the increased utilization of ABR in Europe may be that most European audiologists are also otolaryrengologists.
When the discussion turned to pediatric AIDS, Weimann commented that several pediatric communication disorders such as elective mutism, hysterical aphonia, and pragmatic language disorders or delay appear to be unique manifestations in children with HIV disease. In response to Nary's question about why there is very little reported on communication disorders in HIV-infected children, Weimann speculated that pediatric communication disorders might be overlooked by some physicians who may not have had experience in recognizing the symptoms.
Elise Davis-McFarland, PhD, CCC-SLP, a speech-language pathologist and director of the Communication Sciences and Disorders Program at the Medical University of South Carolina, agreed with Weimann in an overview of her work on pre-linguistic development in HIV-infected and -exposed children. Davis-McFarland is a researcher at the Outpatient Pediatric AIDS Clinic, Medical University of South Carolina Hospital in Charleston, South Carolina. The clinic staff cares for approximately 100 infected and exposed children, and studies the effects of antiretroviral and other drugs on HIV-infected children. Davis-McFarland works with a team that includes three pediatric infectious disease physicians, nurses, a nutritionist, a social worker, a child life specialist, and a psychologist who conducts routine developmental assessments on the children who are cared for at the clinic.
The patient population at her clinic is characterized by a 50/50 gender split, with a racial distribution reflecting the continued racial disparity of AIDS -- 89 percent African-American, and 11 percent White. Davis-McFarland works with infected children from birth through adolescence. Many of these children are from areas throughout the Lowcountry of South Carolina and rural areas adjacent to Interstate Highway 95 (I-95), which is a well-known route for the transport of illegal drugs from Florida to New York.
Davis-McFarland opened her presentation by sharing a statement by Hydea Broadbent, a 13-year-old living with HIV who has become one of this country's most outspoken AIDS advocates. "My life has been a little different than other kids I know. I was born with AIDS." She lauded Hydea's attitude toward her HIV-challenged life as always refreshing and reflective of an optimism that is important in a profession that is always marked by an undercurrent of sadness.
Davis-McFarland discussed her observations that the incidence of pediatric AIDS in patients served by her clinic has been significantly affected by the use of antiretroviral agents by HIV-infected pregnant women. She speculated that whether a child remains HIV-positive may be influenced by the mother's viral load at the time of her pregnancy, and that her infected patients may have been infected earlier rather than later in their mothers' pregnancies.
Davis-McFarland did not have any data on whether other pediatric HIV centers incorporate speech-language pathologists and audiologists into their infrastructures. She pointed out that her clinic does not have an audiologist on its team but rather refers children to audiologists at the hospital's ENT clinic as necessary.
After describing some of the differences between adult and pediatric HIV (Table 2), Davis-McFarland commented on the differences between vertical infection in children and horizontal infection in adults. One hundred percent of the pediatric patients at her clinic were vertically infected. She explained the differences between the incidence of opportunistic infections in adults and children.
|Table 2. Adult vs pediatric HIV|
|Adult HIV||Pediatric HIV|
|Mature CNS||Immature CNS|
|CNS OI frequent||CNS OI infrequent|
|PNS involved||PNS not involved|
|Seizures common||No seizures|
|Brain atrophy||Impaired brain growth|
In treatment-naive adults there is generally a long latency period between the time of infection and the manifestation of the first symptoms. This can take up to ten years or more in some antiretroviral-naive patients. She explained that in adults, HIV manifests itself on a mature central nervous system that results in the common constellation of opportunistic infections and neurological diseases. However, in children, HIV infection is manifested on an immature nervous system that results in a different constellation of HIV-associated diseases.
Davis-McFarland discussed the differences between central nervous system involvement in adult and pediatric AIDS. In adults, brain atrophy is observed, however, in children there is a lack of or impaired brain development -- a difference that can explain the primary cause of communication disorders in HIV-infected children. She reviewed the clinical categories used for describing system suppression as well as signs and symptoms (Table 3) that she would refer to in discussion of her work.
|Table 3. Clinical categories|
|Immunologic categories||N: No signs/symptoms||A: Mild signs/symptoms||C: Severe signs/symptoms|
|1. No evidence of suppression||N1||A1||B1||C1|
|2. Moderate suppression||N2||A2||B2||C2|
|3. Severe suppression||N3||A3||B3||C3|
An important factor contributing to the differences in manifestations of the disease in adults and children is that pediatric HIV is lymphotrophic and neurotrophic. The multinucleated giant cells in the brain may be a reservoir for HIV infection and can damage the developing brain in children if not treated aggressively and immediately.
Davis-McFarland reviewed the common neurological profile of HIV-infected children in her clinic (Table 4) and commented that the extrapyramidal tract signs are similar to cerebral palsy symptoms. Infected children often experience a loss of milestones as a symptom of encephalopathy which can signal HIV progression. She also reviewed the communication disorders common among HIV-infected children (Table 5) and pointed out that delayed language development was more common than uncommon. She noted that the literature on communication disorders in children with HIV indicates that 20 percent of the children may experience dysarthria.
|Table 4. Common neurological profile|
|Table 5. Communication disorders|
Voice disorders such as hoarseness and stridency also occur in young children, especially those with oral and esophageal candidiasis. Phonological (speech) disorders and hearing loss may also occur. Davis-McFarland noted that oral motor dysfunction is a precursor to communication disorders that can be caused by neurological sequelae of HIV infection in children. Some children experience the preservation of primitive oral motor reflexes beyond the normal age of cessation. She also discussed her observations that xerostomia in children is often a side effect of some of their medications. The xerostomia contributes to speech and eating disorders.
She described some of her work in pre-linguistic development in these children and described the Early Milestone Scale 2 (ELMS-2) (Copeland, 1994/revised edition) that provides an assessment of auditory expressive, auditory receptive, and visual language development skills in children. She assessed a variety of developmental behaviors, including: (1) monosyllabic and polysyllabic babbling to the use of short phrases and sentences; (2) auditory receptive behaviors from recognition of environmental sounds to understanding names of common items; and (3) visual language development which provides a social interactive component for the development of language. The ELMS-2 provides subscale scores in each of these three areas as well as a global language score, which is a compilation of the child's score in all three areas.
Davis-McFarland then presented case histories of three children who have been cared for at her clinic (pseudonyms are used for each child):
Bobby's initial score of 80 is within one standard deviation of the mean. But as he gets older, there is a progressive decline in language skills until he is 36 months old, at which time Bobby's scores decline to more than two standard deviations below the mean.
At five months, Andy was fitted with a gastronomy tube for entral feedings and medication intake. He continued to have severe oral motor and swallowing problems. He had severe cognitive deficits and additional signs of neurological dysfunction.
At 15 months, Andy had an MRI indicating bilateral enlargement of the sulci, brain atrophy, and lymphadenopathy.
Davis-McFarland concluded her presentation with some insights that she and her colleagues were learning from the data that they are collecting on these HIV-positive children with communication disorders:
Richard Adler, PhD, CCC-SLP, an Atlanta speech-language therapist in private practice, also studies HIV-associated communication disorders in children and adults. Adler's initial experience with these disorders began in 1987 when he received a telephone call from a friend who complained about the increasing difficulties that he was experiencing in swallowing. Since his friend was hesitant about going to a physician, Adler visited him at home, examined his mouth and throat, and noticed the obvious candida and severe inflammation and swelling in the throat which was later diagnosed as Kaposi's sarcoma (KS). Adler urged his friend to see a physician immediately to receive treatment for the candidiasis. Additionally, Adler's friend had a permanent feeding tube implanted by his physician to bypass the KS obstruction.
Adler, an active member of the Atlanta gay community, then knew very little about AIDS and its relationship to communication disorders. People with AIDS were then subject to considerable discrimination because of their disease and, if they were gay, for their lifestyle. So, Adler began aggressively contacting the relatively small number of physicians in Atlanta who were known at that time to treat people with AIDS to offer his professional assistance primarily to other people affected with HIV-associated oral complications, which at that time were often the primary symptom of the disease.
Because of his interest in AIDS, Adler found that he was receiving a growing number of referrals from physicians who were uncomfortable in even scheduling appointments with patients who were gay or perceived as gay who might be concerned about their HIV risk. Adler said that these physicians generally claimed that they did not have the time for or interest in people with living HIV disease. Although Adler did not have a medical degree, he became one of the primary de facto professionals being consulted for general HIV/AIDS information as well as for professional assistance for HIV-related speech and other oral complications. He also became known in the public school system as an advocate for children with HIV as he increasingly worked with children and educators.
Adler discussed his personal observations on many of the HIV-infected children that are or have been his clients. He discussed his experiences with hysterical aphonia and elective mutism and why he believes that they were caused by or contributed to by the intense discrimination that these children had to face daily. He addressed the children's withdrawal after consistent rejection by the other children, after being denied the use of common bathrooms, or being continually segregated and isolated. Adler also detailed some of his experience with insurance companies in advocating for speech therapy benefits for children with aphonia and mutism, and more recently for what appears to be neurologically-based stuttering.
Adler has collected extensive observational data on his 12 years of experience with pediatric and adult patients that he believes justifies considering some changes in the way certain questions should be directed to people with HIV as well as how some data should be collected and interpreted. He is concerned that his advocacy for gay rights has at times diminished his effectiveness as an advocate on behalf of adults and children with AIDS because of the bigotry and discrimination that continues to lie beneath the misleading superficial appearance of more enlightened acceptance, tolerance, and compassion. ASHA's Weimann praised Adler for his leadership role in ASHA to help raise greater awareness of the complex challenges that HIV disease presents to the association's 96,000 members.
Nary questioned roundtable participants on their observations regarding the central issues that should be addressed jointly by the medical profession, audiologists, and speech-language pathologists. Christensen stated that physicians need to be more concerned about otitis media in children with HIV disease, especially since otitis media is rampant in this population and needs to be diagnosed earlier and treated more aggressively than in non-infected children. She said that there are some data, albeit not conclusive, indicating serious long-term effects of untreated otitis media in HIV-infected children.
Christensen urged consideration of the fact that there will be more and more children surviving HIV disease, but walking around with a 60 decibel hearing loss. She added that in her experience as an audiologist, these children have a higher risk of speech and language development disorders that could affect their entire education.
Bankaitis stressed that the most critical period is the first two years of life. In her experience, some physicians who are not aware of the long-term implications of otitis media in children with HIV disease diminish its seriousness and say, "Well, you have another good ear." She cited data indicating that these children develop speech and language later than non-infected children, and tend to do more poorly in school, and have to be held back a grade.
Christensen was also concerned about the increased risk of CNS degeneration in HIV disease which can also cause cognitive problems and unnecessary hearing loss, which in turn superimposes layer upon layer of additional disadvantages that these children have to face. She reviewed some new pediatric studies that incorporated a cognitive communication component and argued the need to address these issues in all pediatric trials. She specifically addressed the importance of incorporating cognitive and hearing behavior components in all pediatric antiretroviral studies.
Nary suggested a survey of pediatricians and family physicians to learn how they are diagnosing and treating otitis media in children. He said that a goal would be to determine if there is any difference in approach in HIV-positive and HIV-negative children and to try to conduct some retrospective analyses of children's records that might be helpful in determining if and how serious the lack of aggressive treatment of otitis media may be in this population.
Christensen pointed out that the treatment of otitis media in the otolaryngology community is very controversial. This may be in part the result of the impact of managed care on pediatricians, with pediatricians understandably trying to control the treatment of otitis media within their profession and not have the gatekeepers involved in referrals. The audiologists are obviously going to be on the side of the otolaryngologists. So this, like so many other issues, will come down to a question of how cost effective it is to prevent hearing loss in children?
Christensen was further concerned that, especially in HIV, pediatricians and primary care physicians often have been so concerned about life-or-death issues, that they do not understand the impact hearing loss has on a child. She pointed to some innovative treatments in development for otitis media including a new laser treatment that physicians can apply in their office where the laser can make a small incision in the ear, suction the middle ear, and treat the tympanic membrane with a compound that will keep the cut open. This could eliminate the need for general anesthesia for a tube insertion. As these new technologies are developed, there may be a special benefit to include HIV-infected children in the trials. There are also vaccines in development for otitis media, and again, depending on the nature of the vaccine, Christensen said it is not too early to consider enrolling HIV-positive children in any planned Phase III trials.
The ASHA participants reviewed many of the obstacles they have observed in collecting the data necessary to define the extent of communication disorders in people with HIV and to establish more appropriate screening criteria to identify such disorders. Nary and Weimann proposed the formation of an IAPAC/ASHA committee whose responsibilities would include recommendations offered at the October meeting by various participants. Among these proposed responsibilities were:
José Zuniga is the political editor of the Journal and Deputy Director of the International Association of Physicians in AIDS Care.