Pediatric Examination and Board Review (55 page)

Juan is an 18-month-old boy who presents to you for well-child care and is not walking yet. His mother became worried when he was not sitting at 8 months, but her pediatrician at that time said that he would grow out of this. He first rolled over at 5 months, sat alone at 10 months, crept at 12 months, and pulled to stand and cruised at 15 months. He has just started to walk with his hand held but does not walk alone. He prefers to W-sit. He likes to play with toys, especially a busy box, which occupies him for long periods. His mother says he understands what she says but is willful and often noncompliant. He babbles occasionally but has never said “mama.”

Past medical history is remarkable for an uneventful pregnancy except that fetal movements were later and less vigorous per Mom than when she was carrying his older sister. He was delivered uneventfully at term and weighed 3.85 kg. He went home with his mother on the second day and fed well. He has had all of his ageappropriate immunizations and has had no hospitalizations. He has an older sister who is doing well in second grade, but he has an uncle and two cousins with mental retardation.

Juan moved to Chicago from New York City. His mother is single and works at night.

On physical examination, his height, weight, and head circumference are at the 75th percentile. He is not noticeably dysmorphic. His skin has two 2 × 2 cm smooth hyperpigmentations, one on the abdomen and the other on his back. Otherwise, his physical examination is normal. When you or his mother tries to engage him in play, he tries to crawl away. He seems aloof and difficult to engage. He rings a bell you show him and doesn’t want to give it back. You give him some blocks and he puts them all into a cup. You hide a block under a cup and he has no problem finding it. When presented a crayon, he immediately starts scribbling. He heads toward your ophthalmoscope; you softly tell him “no,” causing him to stop briefly but then continue toward it. He picks it up and you hold out your hand and say, “Give it to me” twice. On the second repetition, he hands it over. You ask him to give the cup from among the pile of cubes and he picks up a cube and holds it out to his mother. All of this time, he hasn’t said anything.

SELECT THE ONE BEST ANSWER

1.
What areas are most delayed for Juan?

(A) understanding and using language

(B) fine motor skills

(C) play skills

(D) gross motor skills

(E) social-emotional skills

2.
What test is initially indicated to determine an etiology?

(A) transient otoacoustic emissions and/or automated auditory brainstem response (ABR)

(B) MRI of brain

(C) molecular tests

(D) renal sonogram

(E) all of the above

3.
If Juan has hearing loss, it is least likely to include

(A) sensorineural hearing loss of 80 db

(B) mild conductive hearing loss of 25 db

(C) unilateral hearing loss of 40 db

(D) mixed conductive and sensorineural hearing loss of 60 db

(E) no defects; he has normal hearing

4.
If Juan has bilateral hearing loss, what test is least likely to be helpful?

(A) thyroid

(B) connexin molecular studies

(C) EEG

(D) NF-kappa one functional evaluation

(E) ECG

5.
Management options for children with 90-db hearing loss include all of the following except

(A) amplification

(B) total communication

(C) cochlear implants

(D) oral speech therapy if he has not talked by kindergarten entry

(E) all of the above

6.
When Juan’s mother goes to work, he is left with an uncle who drinks alcohol and becomes aggressive. Which of the following statements is/are true?

(A) because Juan is deaf, he will not be affected by his uncle’s cursing

(B) because Juan has no bruises, he is not being abused

(C) when Juan is aggressive on the playground, it may be related to having his uncle as a caregiver

(D) if Juan is withdrawn in school, it is only because he is beginning to understand he’s deaf

(E) C and D

7.
Helpful strategies when a relative or caregiver has a drinking problem include which of the following?

(A) children’s support groups through Alcoholics Anonymous

(B) ignoring the problem unless there is overt physical abuse

(C) if the caregiver is male, ensure that he is only caring for a male child to alleviate concerns of sexual abuse

(D) foster placement until the mother can arrange for another caregiver

(E) A and D

8.
Supports for successful single parenting include which of the following?

(A) mentoring for the children

(B) ignoring out-of-home behaviors

(C) expecting the teachers to set the best educational goals

(D) respecting a teen’s privacy by never bringing up topics that might make the parent or teen uncomfortable

(E) A and C

9.
Major supports for college education for hearingimpaired teens include which of the following?

(A) Americans with Disabilities Act

(B) National Technical Institute for the Deaf

(C) Gallaudet University

(D) sign language services only available in science classes

(E) A, B, and C

10.
Which of the following statements is true about adults with deafness?

(A) they can only work at workplaces run by deaf individuals

(B) they should not have children unless they marry a hearing adult

(C) they can receive Supplemental Security Disability Income (SSDI) only if they cannot work

(D) if they adopt children, they can only adopt deaf children

(E) none of the above are true

ANSWERS

1.
(A)
Juan’s understanding and use of language is delayed.

2.
(A)
Hearing tests are indicated. Even if a child is too active or immature for play audiometry, transient otoacoustic emission testing or an ABR test will determine if auditory processing mechanisms are intact. Without microcephaly, global developmental delay, macrocephaly, neurologic asymmetry, spasticity, or a movement disorder, MRI is not initially indicated. If there were unexplained global developmental delay in a male, molecular testing for fragile X syndrome would be indicated, but Juan does not have global developmental delay. A renal sonogram would be indicated if there were craniofacial dysmorphism as part of a brachio-oto-renal syndrome.

3.
(C)
A unilateral hearing loss of 40 db would not cause delay in language. This is because intact hearing in the good ear would be adequate for picking up environmental sounds and conversations.

4.
(C)
EEG is least likely to be helpful unless there was a history suggestive of a seizure disorder. Pendred syndrome is associated with hearing loss and hypothyroidism. An ECG is indicated to rule out long Q-T syndrome. Connexin mutations are responsible for an increasing number of nonsyndromic hearing losses.

5.
(D)
Amplification, total communication, and cochlear implants are management options to maximize communication in children with 90-db hearing loss. The critical need is to ensure a communication system so that the child can develop language skills. The choice of what language system (aural or sign) should be discussed with both medical and educational professionals. Speech therapy at kindergarten entry is indicated for children with articulation disorders. If the child has a cochlear implant, a program of aural rehabilitation that includes helping the child understand sound and communicate in words is indicated.

6.
(C)
Children with hearing impairment can learn aggressive behaviors from others. Children with hearing impairment are at risk for abuse, especially if caretakers do not understand that yelling at deaf children is counterproductive. Children with hearing impairment can be bullied by peers. It is critical to assess the safety of the home, school, and community environment. In addition, all children with disruptive behaviors should have a strategy that includes expression of feelings, appropriate social skills, and appropriate consequences for violating social rules.

7.
(A)
Alcoholics Anonymous has support groups for children in families where there are drinking problems. The impact of problem drinking is more than physical abuse. The critical issue is the need for quality adult caregivers and after-school experiences. An important resource would be some of the community organizations providing support after school that would accommodate a child with a hearing disorder.

8.
(A)
Mentoring is a key management strategy for vulnerable children. Resources include YMCA, Scouts, sports teams, and church leaders. Longitudinal studies and population-based adolescent health surveys have demonstrated the critical role of family and mentors in decreasing risk-taking behavior of teens. In large urban school systems, there are gaps in the capacity of educational professionals alone to meet the needs of at-risk children. Though respecting a teen’s privacy is essential for developing trust, parents must undertake the difficult task of communicating both values, expectations, and concerns of problem behaviors even if they cause some discomfort in the teen.

9.
(E)
Interpreter services are available for all classes. The Americans with Disabilities Act requires schools to provide reasonable accommodation to individuals with hearing disorders. Both the National Technical Institute for the Deaf and Gallaudet University are post–high school college programs of excellence for individuals who are deaf or hearing impaired.

10.
(C)
Hearing-impaired individuals can receive SSDI, if they cannot work. The major requirement for SSDI is disability causing an inability to work. The Americans with Disability Act requires accommodations in all workplaces whatever the boss’s hearing status. Individuals who are deaf are free to marry any individual whether hearing impaired or not. Hearing-impaired individuals can adopt any child provided they are able to meet that child’s health, safety, and educational needs. Given the diverse nature of deafness, it should not be assumed that two hearing-impaired parents will have a hearingimpaired child or only choose to have a child without a hearing disorder.

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