Expert Review Panel / Speech and Hearing Domain Community Outreach Response Report

March 22, 2019

The PhenX ERP 7 Community Outreach for new measures and changes to the measures and protocols in the Speech and Hearing Domain was available between February 21 and March 11, 2019.

12 people responded to the outreach e-mail. 10 respondents completed the value section of the measures table. 33 visitors viewed the web page that included the datasheets for review. 1 respondent recommended a protocol for hearing loss in children (see Section I). There were no recommendtions for a protocol for oral/motor cranial nerve examination for adults.

I. Response to the request for hearing loss in children and oral motor/cranial nerve examination for adults Hearing Loss in Children: 1. At the national level, newborns born in the hospital in the US are covered by the Early Hearing Detection and Intervention

(EHDI) program. https://www.asha.org/Advocacy/federal/Early-Hearing-Detection-and-Intervention/ It would likely be useful to cover EHDI’s protocols and steps within PhenX as it may be used more in epidemiologic studies as the EHDI data are starting to come online in usable formats from the individual states.

II. General Comments

1. General recommendation for the ERP: With reference to the below tool, which includes variables related to 'grammar, missing words, nonsense words, comprehension...etc' I have following concerns -

a. age of the subjectsb. their level of education .......etc

IMPT: Therefore, to me I feel - frequently encountered images/pictures could be a better substitute & will consume less time to evaluate majority of subjects!

2. An additional comment that I have is a measure to assess speech sound production across the life-span is needed to be added.

PhenXERP 7 (Speech and Hearing) Outreach Report

III. Protocol Rankings and Comments

The table below provides the measures, votes and comments received from respondents. The abbreviation “NR” stands for “no response.”

New MeasuresMeasure Description of Measurement Protocol Yes No NR Comments

1. Hearing Screener hearScreen USA is a clinically validated, U.S. national hearing screening application (app). The app provides accurate detection of hearing impairment. The user is presented with a 23-step hearing screening test, the Digits in Noise Test (DIN). Completion time is under 3 minutes. If hearing impairment is detected, the app will suggest that the user contact a verified audiologist. Appointments with an audiologist can be made using the app.

9 0 3 1. Limited utility in children. App would only be useful in adults who suspect hearing loss. Could pinpoint frequency-specific hearing loss, possibly from long-term environmental exposure to sound.

2. Trust 23 steps are relevant to age? 3. Measures for auditory processing are needed.

2. Instrumental Assessments of Voice

Laryngeal imaging, acoustics, and aerodynamic analysis protocols with published examples of data collection templates that are used to evaluate voice disorders.

6 2 4 1. I don’t see any value outside clinical utility.2. Who will measure?

3. Auditory-Perceptual Evaluation of Voice

The Consensus Auditory-Perceptual Evaluation of Voice (CAPE-V) indicates salient perceptual vocal attributes; the attributes are: (a) Overall Severity, (b) Roughness, (c) Breathiness, (d) Strain, (e) Pitch, and (f) Loudness. The CAPE-V displays each attribute accompanied by a 100-mm line forming a visual analog scale (VAS). Using a tick mark, the clinician indicates the degree of perceived deviance from normal for each parameter on this scale. For each dimension, scalar extremes are unlabeled.

7 1 4 1. This can be useful for identifying the degree of speech impairment in pre-lingually deaf adults who benefit from hearing aids and/or cochlear implants. Limited utility in post-lingually deaf adults.

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PhenXERP 7 (Speech and Hearing) Outreach Report

Measure Description of Measurement Protocol Yes No NR Comments4. Oral Mechanism/Cranial Nerve Examination for Young Children

This is a thorough examination of a child’s oral structures and functions that are necessary for speech production. It is designed to be administered in conjunction with, or as follow-up to, speech sound testing. Atypical observations may be used to identify the cause of speech disorders. For instance, deficits in neuromuscular functions during this protocol could confirm dysarthria, and deficits in motor planning could confirm apraxia of speech. The orofacial measures include observations of the structures and functionality of the facial muscles, lips, tongue, and jaw.

7 1 4 1. Speech impairment due to structural or functional deficits is not necessarily associated with hearing impairment or loss. I see limited utility with this tool outside those with a speech disorder.

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PhenXERP 7 (Speech and Hearing) Outreach Report

Measure Description of Measurement Protocol Yes No NR Comments5. Phonological Awareness Syllable Repetition Task (SRT) is an 18-item task to

assess the ability to repeat nonwords. Administrator presses the down arrow key to progress through the test stimuli in PowerPoint. Each test item (nonword) will appear on the PC screen in the order on the SRT Scoring Form. The screen should not be visible to the respondent, as the task is purely an auditory one. Every time the recording says a nonword, the respondent is to attempt to say the nonword.

6 3 3 1. I can see the value in those who have a mild or moderate hearing loss. Those who have severe or profound hearing loss will struggle with this test w/out use of hearing aids/CIs.

2. The SRT is a useful measure for identifying children with genetic risk of verbal trait disorders. It was developed for children with limited phonetic inventories or with Speech sound disorders (SSD) to minimize/ eliminate measurement confounds related to speech productionIt uses 18 2-, 3-, and 4-syllable CVs, only /b, d, m, n, ɑ/ sounds that even children with moderate to severe SSD have in inventories. However, I believe the measure is mislabeled. It is not a task of phonological awareness. I believe it should still be labeled as a task to assess nonword repetition. Another concern is that the link to the PPT on the Wisconsin Phonology Project website does not lead to the PPT for retrieving the task.

3. N: We have not found this measure useful; Elision is a better measure of Phonological Awareness.

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PhenXERP 7 (Speech and Hearing) Outreach Report

New ProtocolsMeasure Description of Measurement Protocol Yes No NR Comments

1. Tinnitus The Tinnitus Screener is a six-item interviewer-administered questionnaire that assesses, during the past year, whether tinnitus is present at all, acute or chronic, constant or temporary, intermittent or occasional.

7 2 3 1. Tinnitus can be caused by medical issues and certain medications. It is not limited to hearing domain. I don’t see the value of this screener as it often can be self-diagnosed.

2. I have reviewed the protocols for speech and hearing. I see that the Tinnitus protocol includes the Tinnitus Handicap Inventory. It is generally accepted by professionals/researchers working in this area that the Tinnitus Functional Index is a better instrument. However, many clinicians are more familiar with the THI and it is easier to use/score. If possible, I would recommend that both be included.

3. Need to define the term. Trust this is relevant to age? How will this be identified in children?

2. Voice Impairments The Aging Voice Index (AVI) is a 23-item self-administered questionnaire that asks individuals about the effects of their voice on their lives. The AVI asks the subject to indicate how often each situation occurs in their daily life from the following scale: never, rarely, sometimes, usually, or always. Each item is based on a scale of 0 to 4 and the sum of all items is the total score. The AVI’s maximum score is 92. A higher total score indicates the subject’s voice impairment has a greater impact on the quality of life.

8 0 4 1. Those with hearing and speech impairments and/or loss may experience a reduced or diminished quality of life. To quantify the degree would enable proper identification of the issue and allow sufficient allocation of resources to address it.

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PhenXERP 7 (Speech and Hearing) Outreach Report

Retire MeasureMeasure Description of Measurement Protocol Yes No NR Comments

1. Non-word Repetition Non-word Repetition is an interviewer-administered test that is a part of the Comprehensive Test of Phonological Processing (CTOPP). The respondent listens to a series of nonsense words played on an audio CD and repeats them back to interviewer.

The Phonological Awareness measure (new) collects the same type of information.

Click on this link to view previous protocol.

5 4 3 1. See comments above for 5. Phonological Awareness. I think 3. Auditory-Perceptual Evaluation of Voice is a more useful tool.

2. See comment above (for 5). The replacement task should also be described as Non Word Repetition.(Comment for 5: The SRT is a useful measure for identifying children with genetic risk of verbal trait disorders. It was developed for children with limited phonetic inventories or with Speech sound disorders (SSD) to minimize/ eliminate measurement confounds related to speech productionIt uses 18 2-, 3-, and 4-syllable CVs, only /b, d, m, n, ɑ/ sounds that even children with moderate to severe SSD have in inventories. However, I believe the measure is mislabeled. It is not a task of phonological awareness. I believe it should still be labeled as a task to assess nonword repetition. Another concern is that the link to the PPT on the Wisconsin Phonology Project website does not lead to the PPT for retrieving the task.

3. Since 'nonsense' words may not familiar to respondent the expected recap could be difficult?

4. Nonword repetition is very useful for identifying phenotypes in older individuals. We have found it to be a significant variable in our molecular genetic studies.

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PhenXERP 7 (Speech and Hearing) Outreach Report

Updated ProtocolsMeasure Description of Measurement Protocol Yes No NR Comments

1. Grammatical Impairments – Grammaticality Judgment Task

The Rice Grammaticality Judgment Task is an examiner-administered, 20-item grammar judgment task. The respondent listens to a series of prerecorded sentences (items) and indicates whether or not the sentence is grammatically correct.

Protocol updated with an Ipod™ phone/tablet application.

Click on this link to view previous protocol.

8 1 3 1. I think utility of this tool is limited to those with a mild or moderate hearing loss. Utility of this tool would need to have a very specific purpose.

2. The app is freely available, easy to use, and generates reports A’ scores. The tasks are theoretically-driven and the cutoff scores have been empirically established as sensitive and specific.

3. Although we have not used this specific measure, a test such as this is useful in detecting language impairment.

2. Audiogram Hearing Test

The National Health and Nutrition Examination Survey (NHANES) 2015-2016 audiometry protocol tests the examinee’s hearing threshold (in decibels, dB) at 7 frequencies in a soundproof room. Examinees are asked a series of exclusionary questions, undergo an outer-ear examination (otoscopy), and then have the mobility of their eardrum tested (acoustic immittance). Pure tone signals are then presented to each ear through earphones, and the intensity of the signals is varied until the level is identified at which the person is just able to hear the sound.

Protocol updated from the 2009-2010 NHANES audiometry protocol to the 2015-2016 NHANES audiometry protocol.

Click on this link to view previous protocol.

9 0 3 1. Gold standard for identifying and quantifying degree of hearing loss while minimizing background noise.

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PhenXERP 7 (Speech and Hearing) Outreach Report

Measure Description of Measurement Protocol Yes No NR Comments3. Reading Comprehension

The Passage Comprehension Subtest of the Woodcock-Johnson® Tests of Achievement (WJ IV®) is an interviewer-administered test in which the respondent is asked to read a short passage and identify a missing keyword that makes sense in the context of the passage. WJ IV® can be scored with either the off-line method of estimated scores obtained from the Test Record (Mather & Wendling, 2014) or by using the scoring and reporting online format (Schrank & Dailey, 2014) or with Report Writer.

Protocol updated from The Passage Comprehension Subtest of the Woodcock-Johnson® Tests of Achievement (WJ III®) 2001 to the WJ IV® (2014).

Click on this link to view previous protocol.

7 2 3 1. This is a test of cognitive ability, not hearing or speech.

4. Word Decoding The Test of Word Reading Efficiency–Second Edition (TOWRE-2) is a nationally normed measure of word reading accuracy and speed. The test contains two subtests: the Sight Word Efficiency (SWE) subtest, and the Phonemic Decoding Efficiency (PDE) subtest. The SWE subtest involves presenting the respondent with a list of real printed words. The respondent must accurately read and pronounce as many words as possible in 45 seconds. This subtest evaluates the respondent’s ability to identify sight words.Protocol updated from TOWRE (1999) to the TOWRE-2 (2012). Click on this link to view previous protocol.

7 2 3 1. Sight words are very important to proper speech. This tool can identify speech impairments and indirectly, possible hearing impairments.

2. This measure is very useful as it is a timed measure and often distinguishes affected from non-affected individuals.

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PhenXERP 7 (Speech and Hearing) Outreach Report

Measure Description of Measurement Protocol Yes No NR Comments5. Grammatical Impairments – Clinical Evaluation of Language Fundamentals

The Recalling Sentences subtest of the Clinical Evaluation of Language Fundamentals®, Fifth Edition (CELF®-5) is an interviewer-administered test that determines if a student has a language disorder or delayed language development. It asks a student to imitate sentences presented by the examiner. As the test progresses, the sentences become longer and more complex. Respondents are scored for the number of errors made in each sentence.

Protocol updated from CELF-4 (2003) to CELF-5 (2013).

Click on this link to view previous protocol.

9 1 2 1. It is important to differentiate between language disorders and delayed language development. This can set back those with speech or hearing impairments. Resources can be more readily available with proper identification.

2. Good to update this to the newest version of the CELF.

3. The CELF-5 is an improvement over the CELF-4.

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