JAAA CEU Program

The questions on this page refer to Bogle and Zapala, “Pendular Nystagmus Presenting in Usher Syndrome Type I: A Case Report,” pages 263–269

Learner Outcomes

Readers of this article should be able to:

• Describe pendular low-amplitude high-frequency (PLAHF) nystagmus and etiologies associated with this presentation.

• Apply understanding of typical vestibular diagnostic results to an atypical nystagmus presentation.

CEU Questions

1. Usher syndrome type I is characterized by:

   • Congenital vision loss due to retinitis pigmentosa.

   • Congenital profound sensorineural hearing loss and vestibular arefl exia.

   • Progressive sensorineural hearing and vision loss.

2. Pendular nystagmus presents as:

   • A square wave.

   • A sine wave.

   • A sawtooth wave.

3. Pendular nystagmus in adults is most often associated with:

   • Demyelinating disorders.

   • Acute labyrinthitis.

   • Age-related vision loss.

4. Proteins associated with the PCDH15 gene are responsible for:

   • Neural transmission of sensory information.

   • Appropriate development of type II vestibular hair cells.

   • The links between stereocilia that allow for appropriate mechanotransduction and hair cell synapses.

5. Pendular low-amplitude high-frequency (PLAHF) nystagmus presents with frequency between:

   • 5–10 Hz.

   • 1–5 Hz.

   • 10–12 Hz.

6. Pediatric cases of pendular nystagmus are associated with:

   • High gain within the retina/neural integrator pathway for smooth pursuit.

   • Poor saccade pathway performance.

   • Reduced ability to develop appropriate fi xation pathways.

7. Miners' nystagmus was initially diagnosed as a rotary or pendular nystagmus associated with:

   • Working in insuffi cient lighting for several decades.

   • Chronic exposure to lead and other heavy metals.

   • Hypoxic working conditions and respiratory disease.

8. Treatment for acquired pendular nystagmus due to light deprivation includes:

   • Light therapy.

   • Vestibular rehabilitation.

   • Oculomotor rehabilitation.

9. Patients with Usher syndrome type I may present with:

   • Present caloric responses.

   • Present high frequency vestibular responses.

   • Appropriate gross motor skill development.

10. Pendular nystagmus associated with demyelinating disorders is generally associated with:

    • Symmetric amplitudes for all planes.

    • Symmetric eye-movement trajectories.

    • Intermittent suppression.

Publication History

Article published online:
07 January 2025