What Are Potential Complications of Hearing Aids?

Patient Presentation
A 7-year-old female came to clinic for her health supervision visit. She had a history of recently identified unilateral, moderate, conductive hearing loss that was found during a school hearing screening. She had a history of recurrent otitis media when she was younger. The father stated that they were ordering her a hearing aid but he was concerned about potential complications and wanted to know more about them. The family history was positive for hearing loss in a great grandfather when he was old.

The pertinent physical exam had normal vital signs and growth parameters were in the 50-90%. Her physical examination was normal. The diagnosis of a healthy child with a moderate hearing loss was made. The pediatrician said that he didn’t know exactly what the complications were but not having the hearing aid fitted properly, possible skin infection and wax build up seemed to be likely. He recommended that the father talk again with the audiologist either before or during her next appointment so he understood the common potential problems and how to use the device properly.

Discussion
Hearing loss can range from profound deafness to fairly minor loss. The causes vary based on age, type of loss (sensorineuronal or conductive, about half of hearing loss in children has a genetic cause), degree and audiometric configuration. Sensorineuronal hearing loss involves the cochlea and neural connections to the brain and auditory cortex. Conductive hearing loss involves structures from the external ear to the oval window. Deafness is defined as a hearing loss > 90 dB. Hearing loss can affect the living and learning of children more than many people realize. “A child with a mild hearing loss can miss up to 50% of what is being said in the classroom.” Therefore it is important to screen for hearing loss. A differential diagnosis of hearing loss can be found here.

People with hearing loss or deafness will use different hearing equipment and have different preferences for communication. Use of spoken language is usually a person’s first choice, but they may use lip-reading, a signed language, hearing equipment or a combination of these. Signed languages may need an in-person or video-type interpreter, but there are technologies being developed that are trying to use automated translation. There are also technologies which directly connect hearing aids to radios/televisions/computers or to other equipment within a work environment or home (e.g. telephone, doorbell). It is important to ask the person their communication preferences and provide appropriate communication before, during and after appointments. Other tips for the provider during the visit are the same for any other good communication interaction: get the person’s attention, face the patient without the face or mouth covered, the provider’s face should have good lighting, make sure the topic of conversation is understood, reduce background noise, and continue to try to communicate even when it may be difficult.

Learning Point
Intra-aural or air conduction hearing aids don’t restore sounds but help to magnify the sound and allow people to hear better than they could otherwise. They do not work right away, unlike eyeglasses, as the brain needs to adapt to the improved sound.

Possible complications of intra-aural hearing aids include:

  • Comfort and skin irritation – Ill-fitting devices can cause pain or skin irritation that can be improved with proper fitting
  • Headache and tinnitus often improve with more time as the brain gets used to the improved sound
  • Pruritus caused by cerumen accumulation – keep cerumen moving by cleaning out ears with spray/drops which also help moisturize the canal.
  • Feedback of sound – sounds like whistling/cracking. Usually brief but if consistent or frequent may need an audiologist to evaluate
  • Poor sound quality or improper volume level – may need the audiologist to evaluate, can use a programmable hearing aid, device may need cleaning or batteries
  • Loss of device

Possible complications of bone conduction hearing aids in studies in adults cite <10% for major complications and <25% for minor complications. In a retrospective chart review of pediatric cases, complications were more common than adults with 44% needing revision surgery (e.g. surgical skin interventions, fixture problems), and 53% had minor complications, mainly skin infections and skin overgrowth.

Questions for Further Discussion
1. What are the hearing screening recommendations in your location?
2. What types of hearing screening tests are used and what are their pros/cons?
3. List some genetic syndromes which have hearing loss as a major sign?
4. What are the hearing risks of using earbuds and headphones?
5. How much do hearing aids cost?

Related Cases

To Learn More
To view pediatric review articles on this topic from the past year check PubMed.

Evidence-based medicine information on this topic can be found at SearchingPediatrics.com and the Cochrane Database of Systematic Reviews.

Information prescriptions for patients can be found at MedlinePlus for these topics: Hearing Aids and Hearing Disorders and Deafness.

To view current news articles on this topic check Google News.

To view images related to this topic check Google Images.

To view videos related to this topic check YouTube Videos.

Hearing Aids Side Effects. Hearing Direct. Published September 6, 2017. Accessed October 26, 2020. https://www.hearingdirect.com/blog/hearing-aids-side-effects.html

Chan KH, Gao D, Jensen EL, Allen GC, Cass SP. Complications and parent satisfaction in pediatric osseointegrated bone-conduction hearing implants. Laryngoscope. 2017;127(9):2165-2170. doi:10.1002/lary.26469

Easson A, Walter S. Hearing-impaired young people – a physician’s guide. Clin Med (Lond). 2017;17(6):521-524. doi:10.7861/clinmedicine.17-6-521.

Author
Donna M. D’Alessandro, MD
Professor of Pediatrics, University of Iowa

What Causes Lethargy?

Patient Presentation
A 3 year-old male came to clinic with a 2-day history of cough and runny nose. He had a fever to 38.4°C and complained of a sore throat that worsened with the cough, but denied any other pain. “He’s totally lethargic and is just lying around,” according to his mother. She stated that he was sleeping poorly because of the cough. He was drinking and urinating. He was in daycare. The past medical history was non-contributory.

The pertinent physical exam showed a tired appearing male with temperature of 37.8°C, heart rate of 98, and respiratory rate of 22. HEENT showed copious rhinorrhea, mild erythema of the posterior pharynx without exudate and shotty anterior cervical nodes. His lungs had coarse breath sounds and transmitted upper airway sounds, but he was in no respiratory distress. Neurological examination was normal and the patient was quieter but carried on an appropriate conversation.

The diagnosis of a viral upper airway infection was made. The mother was counseled about symptomatic treatment and reasons to call or return to clinic such as signs of respiratory distress. The patient was tested for novel coronavirus (COVID-19) and eventually was negative also.

Discussion
Lethargy is a common word used to describe a person who is drowsy, sluggish, listless and apathetic. Concentration may be difficult and they may have problems doing simple tasks. Many people actual mean fatigue or lassitude or being more tired when they use the word. In medical terms, lethargy is usually used to describe patients who have some type of excessive tiredness and usually have mental status changes with decreased alertness or arousal. Patients and health care providers both usually qualify the term and give more description to better communicate what is implied when using the term. This is true of many words such as “irritable” or “diarrhea” which again are used frequently but may be less precise as to their meaning, or that have different meaning for laypersons or health care professionals. In medicine, true excessive tiredness can be caused by many problems. History is very important. Is this an acute change with a profound decrease in alertness (e.g. sepsis, meningitis, drug toxicity, etc.), or is this more chronic and the patient and family have noticed a slower onset that is just now causing changes to their activities of daily living but not their mental alertness (e.g. anemia, hypothyroidism, etc.).

Learning Point
The differential diagnosis of fatigue (a review can be found here) and lethargy are large with much overlap. Every organ system can be a potential cause, as the body obviously wishes to conserve energy and rest more when it is ill. Also any extreme state can often cause derangement or distress that manifests in some type of mental status change. One must consider what is meant by the term and especially the onset.

The differential diagnosis for lethargy includes:

  • Acute disease presentations
  • Altered homeostatic states
    • Fever – very common cause
    • Dehydration
    • Malnutrition
    • Insufficient sleep
    • Temperature regulation – too hot or cold
  • Chronic disease of major organ systems and/or organ failure
    • Central nervous system
      • Myasthenia gravis
      • Post-ictal state
      • Unrecognized seizures
    • Cardiac
      • Arrhythmias
      • Congenital heart disease
      • Endocarditis
      • Myocarditis
    • Endocrine
      • Addison’s disease
      • Cushing’s disease
      • Hypothyroidism
      • Hyperthyroidism
    • Gastrointestinal
      • Hepatitis
      • Pancreatitis
    • Hematologic
      • Anemia
    • Pulmonary
      • Respiratory failure
      • Severe asthma
    • Renal
      • Pyelonephritis/Urinary tract infection
      • Renal disease
  • Drug – side effects, abuse
  • Electrolyte abnormalities
    • Hypoglycemia
    • Hyperglycemia
    • Hyperammonemia
    • Metabolic abnormalities
  • Infections – acute and chronic
  • Mental health
    • Anxiety
    • Depression
    • Substance use/abuse
  • Trauma
    • Abuse and neglect
    • Traumatic brain injury

Questions for Further Discussion
1. What other terms are commonly used imprecisely by laypersons and healthcare providers?
2. What causes fever? A review can be found here

Related Cases

To Learn More
To view pediatric review articles on this topic from the past year check PubMed.

Evidence-based medicine information on this topic can be found at SearchingPediatrics.com and the Cochrane Database of Systematic Reviews.

Information prescriptions for patients can be found at MedlinePlus for these topics: Fatigue and Traumatic Brain Injury.

To view current news articles on this topic check Google News.

To view images related to this topic check Google Images.

To view videos related to this topic check YouTube Videos.

Illingworth RS. Common Symptoms of Disease in Children. Blackwell Scientific Publications. Oxford, England. 9th Edit. 1988.

Sheldon SH, Levy HB. Pediatric Differential Diagnosis. Raven Press. New York. 2nd Edit. 1985.

Author
Donna M. D’Alessandro, MD
Professor of Pediatrics, University of Iowa

What Are Potential Problems of Congenital Lung Malformations?

Patient Presentation
A 2-week-old female came to clinic for her health maintenance examination. She was a term infant who had been diagnosed with a congenital pulmonary airway malformation on pre-natal ultrasound in the right chest. The lesion had decreased in size on the last ultrasound before delivery. She was delivered vaginally and had had no problems at delivery, in the hospital or since going home. The pediatric surgeon had seen the patient and wanted to followup in 3 months if the patient remained asymptomatic. Her parents continued to monitor her closely and described “funny breathing” where she will breath slightly more rapidly and then have a short period of not breathing. They do not report labored breathing, and she never has problems with color changes, difficulty feeding or abnormal sounds while breathing.

The pertinent physical exam revealed a 3.980 kg infant with weight gain of 22 grams/day since her last visit. HEENT was normal as was her lung exam. During the examination the parents pointed out the “funny breathing” which was normal periodic breathing.

The diagnosis of an asymptomatic newborn with a prenatally diagnosed congenital pulmonary airway malformation was made. The pediatrician reassured the family about the periodic breathing and reviewed symptoms of respiratory distress and encouraged them to call if she had any problems also with feeding. The patient’s clinical course at her 1-month and 2-month visit showed that she was growing well and continued to be asymptomatic.

Case Image
Figure 128 – AP view of the chest taken on the first day of life shows hazy increased density in the inferior and lateral aspect of the right lung, most probably due to the presence of multiple small cysts in the right lower lobe. The findings were felt to be compatible with a congenital pulmonary airway malformation.

Discussion
Congenital lung malformations (CLM) are commonly identified with prenatal ultrasound and occur in ~1:2400 live births. The natural history shows that lesions may remain the same, enlarge or appear to disappear. There is a wide-range of clinical manifestations. Patients may be asymptomatic or symptomatic at birth and often develop symptoms later.

The overall natural history is difficult to ascertain as there are fewer studies done in older children and adults. Those that are done often lack a good denominator by which to judge the incidence or prevalence. Resection is carried out usually if a patient is symptomatic, but in patients who are asymptomatic the decision can be more difficult. Timing of resection for asymptomatic patients is debated and usually occurs between 6 month – 2 years and try to spare as much tissue as necessary. Main reasons for early treatment of CLMs is the potential for continued or compensatory lung development and growth. Alveolar units continue to develop until age 2 and there can be increased size of existing alveoli before and after this age. Within the alveoli there are changes that can occur up to age 4-5 years. Other studies show many children becoming asymptomatic but again there are complicating issues with these studies.

Common types of CLMs include:

  • Bronchogenic cysts
    • Lesions arise from the bronchial tree usually around the mediastinum, lined with respiratory epithelium that are mucus-filled, may have a component of cartilage
    • Lesions are single or multicystic
    • Usually resected
  • Bronchopulmonary sequestration (BPS)
    • Lesion is pulmonary tissue that does not connect to the tracheobronchial tree and whose arterial blood supply is systemic (not pulmonary)
    • Can be located extra-lobar or intra-lobar
    • Can also be a hybrid lesion with CPAM (see below)
    • Usually resected
  • Congenital pulmonary airway malformation (CPAM) – 1 in 10,000 live births
    • Lesions are multicystic, of various sizes and locations
    • They can be unilobar, bilobar, multifocal and even bilateral
    • Can also be a hybrid lesion with BPS
    • Usually resected
  • Congenital lobar emphysema (CLE)
    • Pulmonary lobe(s) are overdistended as it is thought that there is a localized catilaginous deficiency which causes local bronchomalacia and overdistention
    • Airway collapse with expiration can cause air trapping distally leading to overdistention
    • Often resected but depending on the lesion they may be followed conservatively

Learning Point
CLM outcome is correlated primarily to lesion size. Patients may have outcomes from intra-natal fetal death to being asymptomatic for long periods of time.

Potential problems and complications of CLMs include:

  • Fetal demise
  • *Respiratory problems – tachypnea, oxygen requirement, respiratory collapse
  • *Lesion expansion – mass effect and/or loss of pulmonary tissue
  • *Infection – pneumonia, cyst infection
  • *Malignancy – confused with a primary malignancy (i.e. pleuropulmonary blastoma (extremely rare and seen almost exclusively in young children, bronchioalveolar carcinomas) or potential transformation to a malignancy
  • Cardiac issues – due to mass effect or in BPS high output cardiac failure can occur if a large arterial blood vessel serves it
  • Hemorrhage
  • Spontaneous pneumothorax
  • Resection problems – chest wall deformities, scoliosis, nerve damage, scarring problems

* issues commonly cited as reasons for resection

Questions for Further Discussion
1. What is in the differential diagnosis of respiratory distress? A review can be found here
2. What causes respiratory failure? A review can be found here
3. What are indications for pediatric surgery referral?

Related Cases

    Disease: Congenital Pulmonary Airway Malformation | Birth Defects

To Learn More
To view pediatric review articles on this topic from the past year check PubMed.

Evidence-based medicine information on this topic can be found at SearchingPediatrics.com and the Cochrane Database of Systematic Reviews.

Information prescriptions for patients can be found at MedlinePlus for these topics: Birth Defects and Topics.

To view current news articles on this topic check Google News.

To view images related to this topic check Google Images.

To view videos related to this topic check YouTube Videos.

Boucherat O, Jeannotte L, Hadchouel A, Delacourt C, Benachi A. Pathomechanisms of Congenital Cystic Lung Diseases: Focus on Congenital Cystic Adenomatoid Malformation and Pleuropulmonary Blastoma. Paediatric Respiratory Reviews. 2016;19:62-68. doi:10.1016/j.prrv.2015.11.011

Hall NJ, Stanton MP. Long-term outcomes of congenital lung malformations – Semin Pediatr Surg. 2017 Oct;26(5):311-316. Accessed October 6, 2020. https://pubmed.ncbi.nlm.nih.gov/29110827/

Leblanc C, Baron M, Desselas E, et al. Congenital pulmonary airway malformations: state-of-the-art review for pediatrician’s use. European Journal of Pediatrics. 2017;176(12):1559-1571. doi:10.1007/s00431-017-3032-7

Author
Donna M. D’Alessandro, MD
Professor of Pediatrics, University of Iowa

How Long Do You Monitor Neonates for Neonatal Abstinence Syndrome (NAS)?

Patient Presentation
A term gestation, newborn female was delivered vaginally to a G2P2 female with a history of heroin addiction. She had been on methadone treatment for 11 months and was doing well in treatment. She had prenatal care and her screening tests were negative. The intrapartum history was non-contributory and at delivery her Apgar scores were 8 and 9. The family history showed poly-drug addiction in the distant past, with only heroin addiction recently. The mother was getting regular mental health treatment. The parents were not married but both were employed and had 2 children by previous relationships in the household. The father had alcohol and tobacco use and no history of other substance use or abuse.

The pertinent physical exam at 30 minutes of life revealed a normal appearing female. Her weight was 3180 grams (25%), length 53 cm (50%) and head circumference of 34.0 cm (50%). She had some molding of her head. Normal red reflexes bilaterally. Heart was regular rate and rhythm without murmur. Lungs were clear for age. She had a 3-vessel cord. Neurologically she had good tone and strength.

The diagnosis of a healthy female was made. The parents agreed to all routine neonatal care at birth and she was monitored using the Finnagan scoring system. The mother was already aware that a cord drug screen would be sent because of the drug use which eventually was negative for any drugs that were not expected. The family also met with the social worker who did not identify any additional specific needs. The patient’s clinical course over the next 7 days did show intermittent elevated Finnagan scores but none were elevated serially so that pharmacological treatment was not needed. The patient was discharged and followed by the local family physician.

Discussion
Neonatal abstinence syndrome (NAS) occurs in the first few days of life and is a complex withdrawal syndrome. The newborn is abruptly cut-off from their exposure to licit or illicit drugs that the mother is chronically consuming and which were being transmitted through the placenta to the fetus. “NAS is a highly variable and severe condition; it may be associated with central and autonomic nervous system dysfunction … and gastrointestinal disorders.” It can cause significant morbidity but is rarely fatal.

Fetal exposure has been increasing in the United States. In 2011-12, almost 5.9% of pregnant women were identified as drug abusers with the highest proportion being in the adolescent age group (15-17 years, 18.3%). A variety of drugs can cause it including opioids, methamphetamines and psychotropic agents. Fetuses exposed to opioids can have prematurity, intrauterine growth restriction, low birth weight, congenital anomalies and NAS. Transplacental passage of opioids increases with increasing gestational age and can also pass into the breast milk. Maternal treatment for drug addition can include long-acting opioids and other medications such as methadone or buprenorphine. Neonates of mothers using buprenorphine usually have milder post-natal courses than those treated with methadone, which is thought to be due to combinations of environment, physiologic and genetic factors.

NAS has variable onset, presentation and severity. Early onset (within 48 hours) is common for heroin, morphine or anti-depressant exposure. Late onset (after 48 hours) is common for methadone or buprenorphine exposure.
Common symptoms include:

  • Central nervous system excitement – irritability, inconsolable crying, jittery, tremors, hypertonicity, seizures
  • Central nervous system depression – lethargy, poor suck
  • Autonomic nervous system – diaphoresis, temperature instability particularly hyperthermia, mottling, frequent sneezing and yawning, poor sleep
    Hyperphagia is common with poor weight gain because the infants have high caloric needs (up to 150-250 kcals/kg/day).

  • Cardiac – tachycardia, hypertension
  • Gastrointestinal – diarrhea, emesis, electrolyte abnormalities, dehydration

Treatment includes keeping the infant with the family as appropriate, in a quiet, low-light environment with gentle handling. Non-nutritive sucking and breastfeeding (if not contraindicated) can also help the infant. Small, frequent amounts of liquid nutrition often help the infant. The Finnegen scoring system is one system designed to be used for monitoring term infants. Patients are serially monitored and scored and can assist in determining if additional interventions are needed including more frequent monitoring, pharmacological initiation, duration and weaning. Pharmacological treatment extends the usual hospital stay significantly with a mean stay of around 3+ weeks.

Learning Point
According to the American Academy of Pediatrics, “An infant born to a mother on a low-dose prescription opiate with a short half-life (eg, hydrocodone; average half-life, 4 hours) may be safety discharged if there are no signs of withdrawal by 3 days of age, whereas an infant born to a mother on an opiate with a prolonged half-life (eg, methadone) should be observed for a minimum of 5-7 days.

Questions for Further Discussion
1. Under what conditions would breastfeeding be encouraged and discouraged in mothers using licit or illicit substances?
2. What is the protocol for pharmacological intervention in NAS at your institution?
3. In your local environment, what is the role of the Department of Human Services, Social work and similar services in the care of a family with a substance exposed infant?
4. What are the potential long-term problems of in-utero exposure to illicit substances?

Related Cases

To Learn More
To view pediatric review articles on this topic from the past year check PubMed.

Evidence-based medicine information on this topic can be found at SearchingPediatrics.com and the Cochrane Database of Systematic Reviews.

Information prescriptions for patients can be found at MedlinePlus for these topics: Pregnancy and Opioids and Pregnancy and Drug Use and Opioid Misuse and Addiction.

To view current news articles on this topic check Google News.

To view images related to this topic check Google Images.

To view videos related to this topic check YouTube Videos.

Hudak ML, Tan RC, Drugs TCO, Newborn TC on FA. Neonatal Drug Withdrawal. Pediatrics. 2012;129(2):e540-e560. doi:10.1542/peds.2011-3212

Raffaeli G, Cavallaro G, Allegaert K, et al. Neonatal Abstinence Syndrome: Update on Diagnostic and Therapeutic Strategies. Pharmacotherapy: The Journal of Human Pharmacology and Drug Therapy. 2017;37(7):814-823. doi:10.1002/phar.1954

Tran TH, Griffin BL, Stone RH, Vest KM, Todd TJ. Methadone, Buprenorphine, and Naltrexone for the Treatment of Opioid Use Disorder in Pregnant Women. Pharmacotherapy: The Journal of Human Pharmacology and Drug Therapy. 2017;37(7):824-839. doi:10.1002/phar.1958

Isemann BT, Stoeckle EC, Taleghani AA, Mueller EW. Early Prediction Tool to Identify the Need for Pharmacotherapy in Infants at Risk of Neonatal Abstinence Syndrome – Pharmacotherapy: The Journal of Human Pharmacology and Drug Therapy. 2017;37(7):840-848. Accessed September 28, 2020. https://accpjournals-onlinelibrary-wiley-com.proxy.lib.uiowa.edu/doi/10.1002/phar.1948

Holmes AP, Schmidlin HN, Kurzum EN. Breastfeeding Considerations for Mothers of Infants with Neonatal Abstinence Syndrome. Pharmacotherapy: The Journal of Human Pharmacology and Drug Therapy. 2017;37(7):861-869. PubMed. Accessed October 6, 2020. tps://pubmed.ncbi.nlm.nih.gov/28488805/

Author
Donna M. D’Alessandro, MD
Professor of Pediatrics, University of Iowa